THE  LIBRARY 
OF 

THE  UNIVERSITY 

OF  CALIFORNIA 

LOS  ANGELES 


THE    DISEASES    OF 
CHILDREN 

A  WORK  FOR  THE  PRACTISING  PHYSICIAN 


EDITED    BY 

Dr.  M.  PFAUNDLER,  Dr.  A.  SCHLOSSMANN, 

Professor  of  Children's  Diseases,  and  Director  of  the  Professor  of  Children's   Diseases  and   Director  of  the 

Children's  Clinic  at  the  University  of  Munich.  Children's  Clinic  at  the  Medical  Academy 

in  Dusseldorf. 

ENGLISH    TRANSLATION 

EDITED    BY 

HENRY  L.  K.  SHAW,  M.D.,  LINN^US  La  FE'tRA,  M.D., 

Albany,  N.  V.,  Clinical   Professor  Diseases  of  Children,  New- York,  N.Y.,  Instructor  of  Diseases  of  Children,  Colum- 
Albany  Medical  College  ;    Physician-in-Charge  bia  University  ;   Chief  of  Department  of  Diseases  of 

St,  Margaret's   House  for  Infants,  Children,  Vanderbilt  Clinic  ;  Ass't  Attending 

Albany.  Physician  to  the  Babies'  Hospital. 

WITH   AN    INTRODUCTION    BY 

L.   EMMETT    HOLT,   M.D., 

New  York,  N.  Y.,  Professor  of  Pediatrics,  Columbia  University 


/N    FOUR     FOLUMES 

Illustrated  in   black  and  n-hite  and  in   colors  by  6l  full-page  plates 
and  4J0  tixt  cuts. 

VOL.  II. 


PHILADELPHIA  ©  LONDON 
J.    B.    LIPPINCOTT    COMPANY 


Copyright,  1908 
By  J.  B.  LippiNcoTT  Company 


Electrotyped  and  Printed  by  J.  B.  Lippincott  Company 
The  Washington  Square  Press,  Philadelphia,  U.S.A. 


VOL.  II 

Special  Part 


Table  of  Contents 


VOLUME  II. 

PAOK 

Diseases  of  the  Newhohn I 

Dr.  W.  Knopi'elmacher,  Vienna;  translated  by  lir.  A.  S.  Maschke,  Cleveland,  O. 

PrEMATFRITY  .\ND  CoNGENIT.tL  Debility 81 

Dr.  O.  Rommel,  .Munich;  translated  by  Dr.  A.  S.  Masclike,  Cleveland,  (). 

AsPHYXi.\  .\ND  Atei,ect.\sis 99 

Dr.  O.  Rommel,  Munieli;  tran.slated  by  Dr.  A.  S.  Masclike,  Cleveland,  O. 

ScLERrEDEM.V  .VND  SCLEREMA 105 

Dr.  O.  Rommel,  Munich;  translated  by  Dr.  A.  S.  Maschke,  Cleveland,  (). 

Diseases  of  Puberty Ill 

Professor  C.  Seitz,  Munich;   translated  by  Dr.  John  Howland,  New  York,  N.  Y. 

Diseases  of  the  Blood  and  of  the  Blood-preparing  Organs 131 

Dr.  A.  Japha,  Berlin;  translated  by  Dr.  Edward  F.  Wood,  Wilmington,  N.  C. 

HiEMORRHAGIC  AFFECTIONS 1  09 

Dr.  R.  Heoker,  Munich;  translated  by  Dr.  Edward  F.  Wood,  Wilmington,  N.  C. 

Infantile  Scurvy 186 

Professor  W.  von  Starck,  Kiel;  translated  by  Dr.  Charles  K.  Winne,  Jr.,  Albany,  K.  Y. 

Rachitis 196 

Professor  W.  Stoltzner,  Halle;  translated  by  Dr.  Charles  K.  Winne,  Jr.,  .\lbany,  X.  V. 

Di.\betes  Mellitus 219 

Professor  C.  von  Noorden,  Vienna;    translated  by  Dr.  Andrew  Macfarlane,  Albany, 
N.  Y. 

Diabetes  Insipidus 225 

Professor  C.  von  Noorden,  Vienna;    translated  liy  Dr.  Andrew  Macfarlane,  Albany, 
N.  Y. 

Scrofula 227 

Dr.  B.  Salge,  Dresden;  translated  by  Dr.  Godfrey  R.  Pisek,  New  York,  N.  Y. 

Measles 243 

Dr.    P.   Moser,    Vienna;     translated    by    Dr.    Hnmld    Parsons,   M.R.C.P.,    (Londonl, 
Toronto,  Canada. 

Scarlet  Fever 268 

By  Dr.  C.  von  Pirquet.  Vienna,  and  Dr.  B.  Schick.  Vienna,  translated  by  Dr.  Ij^aac  A. 
Abt,  Chicago,  111. 

Rotheln — German  Measles — Rubella 321 

Professor  J.  von  B('ikay,  Budapest;   translated  by  Dr.  John  Ruhrah,  Baltimore,  Md. 

V 


vi  TABLE    OF   CONTENTS 

PAGE 

Dukes'  "  Fourth  Disease  " 326 

Professor  J.  von  Bokay,  Budapest;   translated  by  Dr.  Jolin  Ihilirah,  Baltimore,  Md. 

Vakicella 330 

Dr.  N.  Swoboda,  Vienna;   translated  by  Dr.  John  Ruhrali,  Baltimore,  Md. 

Vaccination 348 

Dr.  L.  Voigt,  Hamburg;  translated  by  Dr.  John  Huhrah,  Baltimore,  Md. 

Diphtheria 355 

Dr.  J.  Tnunpp,  Mimich;  translated  by  Dr.  Alfred  Hand,  Jr.,  Philadelphia,  Pa. 

Mumps — Epidemic  Parotitis 419 

Dr.  E.  Moro,  flratz;  translated  by  Dr.  Frank  X.  Walls,  Chicago,  lU. 

Typhoid  Fever — Abdomixal  Typhus 426 

Professor  R.  Fisclil,  Prague;  translated  by  Dr.  Frank  X.  Walls,  Chicago,  111. 

Dysentery 444 

Dr.  J.  Langer,  Prague;  translated  by  Dr.  Frank  X.  Walls,  Cliioago,  HI. 

Influenza 450 

Dr.  J.  H.  Speigelberg,  Munich;  translated  by  Dr.  Henry  L.  K.  Shaw,  Albany,  X.  Y. 

Whoopixg-Cough 45S 

Dr.  R.  Neuratli,  \'icnna;  translated  by  Dr.  Frank  X.  Walls,  Chicago,  111 

Acute  AnTim.AR  Rhet':matism 4S1 

Dr.  J.  Ibrahim,  Heidelberg;  translated  by  Dr.  Godfrey  R.  Pisek,  New  York,  X.  Y. 

Syphilis 500 

Dr.  C.  Hochsinger,  \icnna;  translated  by  Dr.  Joseph  Brennemann,  Chicago,  lU. 

Tuberculosis.  .    568 

Professor  A.  Schlossmann,  Diisseldorf;  translated  by  Dr.  Alfi;pd  F.  Hess,  New  York, 
N.  Y. 

Index 613 


List  of  Illustrations 


VOLUME  II. 

PACK 

1 .  Injury  to  Soft  Tissues  of  the  Head 2 

2.  Schematic  Cross-section  through  CephaiuCmatoma 6 

3.  Br-^chial  Plexus H 

4.  Dl\gr.\mmatic  View  of  Fcetal  Circulation 27 

5.  Hernia  of  the  Umbilical  Cord 32 

6.  Persistence  of  Ductus  Omphalo.mesentericus 35 

7.  Tet.vnus  Neonatorum 48 

8.  Tetanus  Neonatorum 48 

9.  Tetanus  Neonatorum 49 

10.  Mort.\lity  Table  for  Premature  Infants 90 

11.  Mort.vlity  Curve  of  First  Month  of  Life 91 

12.  Finkelsteln's  Incub.^^tor 93 

13.  Rommel's  Incub.\tor 94 

14.  Pl.\N  of  iNCUB.iTOR  RoO.M  .\T  THE  EsCHERICH  ClINIC 95 

15.  L'-sh.vped  Hot  W.4.ter  Bottle  for  Prem.\ture  Inf.\nts 96 

16.  Feeding  Glass  for  Prem.iture  Infants 97 

17.  PuLMON.4.RY'  ATELECT-iSIS  IN  THE  NEWBORN 100 

18.  sclercedema  in  the  newborn 106 

19.  Sclerema  of  the  Cutis  in  the  Newborn 108 

20 .  Curve  Showing  Blood  Composition 132 

21 .  An.emi.v  Pseudoleuk,emic.\ 142 

22.  An.eml\  Pseudoleuk.emic.\ 143 

23.  Acute  .vnd  Chronic  Leuk/EMm. 144 

24.  Chronic  Leuk,emia 162 

25.  Chronic  Leuk.emia 163 

26.  pseudoleuicemia 165 

27.  REL.A.TI0N  of  Figures  in  Noril\l  .\nd  P.\thologic  Blood 166 

28.  PuRPUR-i  Simplex 174 

29.  PURPUR.\   RHEUM.iTICA 176 

30.  PuRPUR-i  H.EMORRH.^GIC.i 177 

31 .  R.iCHiTis '. 197 

32.  R.\chitic  Scoliosis 199 

33.  Rachitic  Deformity'  of  the  Thorax 201 

34.  Deformity  of  the  Thor-\x,  Pelvis  .\nd  Extremities 202 

35.  r.a.chitic  be.a.d-like  fingers 203 

36.  Rachitic  X-shaped  Legs 204 

37.  Section  through  Epiphyseal  End  of  Tibli 209 

38 .  OsTEOlUiACL*. 21 1 

39.  R.\CHiTic  Funnel  Bre.^st 216 

40.  Epstein  Rocking  Ch.\ir 218 

41 .  Measles  without  Conjunctr-itis 245 

42.  Temper.\ture  Curve  in  Me.\sles 246 

43.  Temper.\ture  Curve  in  Me.\sles 247 

44.  Temper.\ture  Curve  in  Me.\sles 248 

45.  Temper.\ture  Curve  in  Me.vsles 249 

vii 


viii  LIST   OF   ILLUSTRATIONS 

PAGE 

40.   TEMPER.\TrRE  CrRVE  IX  Measles 250 

47.  Typical  Tempekatuke  Cuuve  in  .Scarlet  Fever 269 

48.  FAV0R.4.BLE  RESULT  IN  A  ScARLET  FevER  TONSILLITIS 275 

49.  Necrosis  of  Throat 275 

50.  Typical  Temperature  Curve  in  Scarlet  Fever 283 

51 .  Temper.\ture  Curve  in  Scarlet  Fever 284 

52.  Atypica-l  Temper.\turb  Curve  in  Scarlet  Fever 285 

53.  Continued  Fever  in  Sc.a.rlet  Fever 286 

54.  Temper-\ture  Curve  in  Scarlet  Fever 298 

55.  Temper.\ture  Curve  in  Scarlet  Fever 301 

56.  Temper.vture  Curve  in  Sc.\rlet  Fever 303 

57.  Temper-A-TURE  Curve  in  Sc.4.rlet  Fever 304 

58.  Teiiper-vture  Curve  in  Scarlet  Fever 305 

59.  Temper.\ture  Curves  in  Scarlet  Fever 306 

60.  Temper-^ture  Curve  in  Scarlet  Fever 307 

61 .  Effect  of  Streptococcus  Serum  on  the  Temperature  in  Scarlet  Fever 317 

62.  Section  Through  Varicella  Vesicle '. 336 

63.  H.emorrhagic  Variola 337 

64.  Scars  after  Varicella 338 

65.  Varicella  En.vnthem  in  Mouth 339 

66.  VARICELL.V  EnANTHEM  IN  VuLVA 340 

67.  Variola-like  Eruption  in  Varicell-\ 343 

68.  Gangrenous  Varicella 344 

69.  Variol.\-like  Eruption  after  E.\ting  Decayed  Fruit 346 

70.  Vaccinal  Changes  in  Corneal  Cells 349 

71 .  Primary  Vaccin.^tion  Pustule 350 

72.  Revaccin.\tion  Pustule 350 

73 .  Secondary  Vaccinia 351 

74.  Vaccination  Instruments 352 

75.  Inoculation  Points  in  a  Calf 353 

76.  Chart  Showing  Diphtheri.a^  Mortality 356 

77.  Chart  Showing  Mortality  from  Infectious  Diseases 356 

78.  Chart  Showing  Diphtheria  Mortality 356 

79.  Chart  Showing  Diphtheria  Mortality 356 

80.  Chart  Showing  Frequency  of  Clinical  Forms  of  Diphtheria 357 

81 .  Diphtheria  Bacilli  from  Membrane 358 

82.  Diphtheria  Bacilli  Mixed  with  Cocci 358 

83.  Diphtheria  Bacilli  from  Bouillon  Culture 358 

84.  Temper,\ture  Chart  of  Pharyngeal  Diphtheria 368 

85.  Temperature  Cil^rt  of  Pharyngeal  Diphtheria 369 

86.  Temper-vture  Ch-\rt  of  Progressive  Diphtheria 370 

87.  Temperature  Chart  of  Progressive  Diphtheria 371 

88.  Fibrinous  Exudate  in  the  Larynx 375 

89.  Temper-\^ture  Chart  of  Diphtheria  GRAvissiiLi 378 

90.  Temper.\ture  Chart  of  Diphtheria  Gravissima 379 

91 .  Loffler's  Forceps 390 

92.  Inspection  of  Pharynx  from  Above 401 

93.  Injection  of  Serum  in  the  Later.\l  Chest  Wall 405 

94.  Chart  Sno-mNG  Effects  of  Antitoxin 406 

95.  Ste.am  Room  for  Diphtherl^^ 411 

96.  Ste.\m  Room,  Improvised 412 

97.  Intubation  Set  with  Ebonite  Tubes 414 

98.  Manner  of  Holding  Child  during  Intubation 415 

99.  Intubation — Step  1 416 

100.  Intubation — Step  2 416 

101 .  Intubation — Step  3 416 

102.  Left-sided  Mumps 420 


LIST  OF  ILLUSTRATIONS  ix 


psr.K 


103.  TmiPERATrnE  Chart  in  Mtbips  421 

104 .  Temperature  Chart  ix  Typhoid  Fever 429 

10.5.   TEMPER.\TrRE  Chart  IX  Relapses  of  Typhoid  Fever 436 

106.  IxFLiExzA  Bacilli 451 

107.  SptTUM  IX  WHoopixG-corGH 46.3 

108.  Temperatire  Chart  ix  Whoopixg-cough 466 

109.  Facies  Pertussea 466 

110.  Chronic  Articvlar  Rheuslitism 493 

111.  Chroxic  Articul.\r  Rheum-vtism 494 

112.  Chroxic  Articul-vr  Rheumatism 495 

113.  Chroxic  Arthritis  after  Measles 496 

114.  Primary  Chroxic  Arthritis 497 

115.  Primary  Chroxic  Arthritis 498 

116.  Sectiox  of  Luxg  of  Syphilitic  Fcetus 509 

117.  lL\cuL.iR  Syphilides  ox  the  Face 514 

118.  Diffuse  Crusty  Syphilide  ox  the  Face 516 

119.  Syphilitic  Pemphigus  ox  the  Feet  of  a  Xewborx  Babe 517 

120.  Vertical  Sectiox  of  Syphilitic  Bleb 517 

121 .  Syphilitic  Papules  ox  the  Forehead 518 

122.  >L\cuLOP.\^PTiL.ui  Syphilide 520 

123.  Papulopustular  Syphilide 521 

124.  Sagitt.\l  Sectiox  of  Kxee-Joixt 522 

125.  RoxTGEx  Picture  of  Upper  Extremity  of  Syphilitic  Fcetus 523 

126.  Roxtgex  Picture  of  Lower  Extremity  of  Syphilitic  Fcetus 523 

127.  Roxtgex  Picture  of  Syphilitic  Child  with  Par.\lysis  of  Arm 525 

128.  Roxtgex  Picture  of  Right  H.vxd  of  Syphilitic  Ixf.\xt 526 

129.  Roxtgex  Picture  of  Left  Arm  of  Syphilitic  Ixfant 526 

130.  Secttiox  through  Muscle  of  Syphilitic  Ixfaxt 527 

131 .  Pseudoparalysis  of  Right  Arm  of  Syphilitic  Ixfaxt 529 

132.  Syphilitic  Hydrocephalus 535 

133.  Syphilitic  Hydrocephalus 536 

134.  Syphilitic  Hydrocephalus 537 

135.  Syphilitic  Laryxx  ix  a  Fifteex-Months-Old  Infant 542 

136.  Tuberculosis  of  the  Ixtima  of  a  Vein 580 

137.  Tubercle  Bacilli  ix  the  Luxg  Tissue 580 

138.'  Advanced  Ptjlmoxary  Tuberculosis 582 

139 .  Posterior  View  of  Same  Case 582 

140.  Pulmonary  Tuberculosis 583 

141 .  Pulmonary  Tuberculosis 583 

142.  Temper,\ture  Ch.\rt  Showing  Tuberculin  Reaction 587 

143.  Temperature  Chart  Showing  Tuberculin  Reaction 587 

144.  Temper-\ture  Ch-irt  Showing  Tuberculin  Re.\ctiox 587 

145.  Temper.\ture  Chart  Showing  Tuberculin  Re.\ction' 588 

146.  Temperature  Ch.vrt  Showlxg  Tuberculin  Reaction 588 

147.  Temper-vture  Chart  Showing  Tuberculin  Reaction 588 

148.  Temperature  Ch.\rt  ix  a  Case  of  Inf.vntile  Tuberculosis 594 

149.  Bronchlvl  .\nd  Other  Lymph-glands  Mainly  Affec:ted  in  Tuberculosis 601 


LIST    OF    PLATES 

6.  Dise.\ses  of  the  N.wel 36 

7.  D1SE.VSES  OF  THE  Blood 141 

8.  IxF.vNTiLE  Scurvy 186 

9.  Radiogr-\ph  in  Ixf.\^xtile  Scurvy 195 

10.  R.vdiogr-^ph  in  Rachitis 218 


X  LIST  OF  ILLUSTRATIONS 

PAGE 

11.  Scrofula 234 

12.  Measles 243 

13.  (a)  Eruption  of  Measles;  (6)  Erythema  Infectiosum 251 

14.  Koplik's  Spots 259 

15.  Eruption  of  Scarlet  Fever 276 

16.  Eruption  of  Scarlet  Fever 286 

17.  (a)  Gangrene  after  Scarlet  Fever;   (b)  Enanthem  on  the  Intestinal  Mucous 

Membrane 296 

18.  (a)  Varicella  on  Hand  and  Arm;     (h)  Desquam-voon  i.\  Scarlet  Fever;     (c) 

Softened  Ly.mph-glands  after  Scarlet  Fever 335 

19.  («)  Variola;   {b)  Varicella 347 

20.  Diphtheria 370 

21.  (a)  Tonsillar  Diphtheria;    (6)  Diphtheri.\  of  the  Lips;    (c)  Diphtheru.  of  the 

Uvula 377 

22.  (a)  Diphtheri.^  of  a  Bronchus;    ((;)  Interstitial  Myocarditis  in  Diphtheri.\; 

(c)  Degeneration  of  Kidney 385 

23.  Dysentery 445 

24.  Syphilitic  Onychia;  Skin  Lesions  in  Hereditary  Syphilis 503 

25.  Skin  Lesions  in  Syphilis 513 

26.  Pemphigus  of  Syphilitic  Origin 522 

27.  Skin  Lesions  in  Hereditary  Syphilis 541 

28.  Skin  Lesions  in  Hereditary  Syphilis 550 

29.  RoNTGEN  Pictures  in  Hereditary  Syphilis 560 

30.  Pulmonary  Tuberculosis  in  a  Girl 576 

31.  Miliary  Tuberculosis  of  tfe  Lungs  a.^d  Bronchial  Glands 589 

32.  Miliary  Tuberculosis  and  Tuberculous  Bronchial  Glands 600 

33.  Sections  from  Tuberculous  Lungs 607 


The  Diseases  of  Children 


DISEASES  OF  THE  NEWBORN 

BY 

Dr.  W.  KNOPFELMACHER,  of  Vienna 

translated  by 
Dr.  a.  S.  MASCHKE,  Cleveland,  O. 


I.     BIRTH  INJURIES 

The  foetus  is  exposed  to  many  injuries  during  its  passage  into  the 
world  besides  those  directly  dependent  on  the  act  of  labor.  A  part  of 
these  injuries  are  attributable  to  the  physician  or  nurse,  some  are  de- 
pendent on  a  disproportion  between  the  size  of  the  child  and  the  width 
of  the  pelvis,  others  are  due  to  disturbance  of  the  placental  blood  supply 
from  contraction  of  the  uterus  and  compression  of  the  cord. 

The  physician  should  make  a  careful  examination  of  every  new- 
born infant  to  ascertain  whether  any  birth  injuries  have  occurred. 
The  majority  of  injuries  are  found  in  cases  where  no  artificial  aid  has 
been  employed.     Dittrich  classifies  birth  injuries  as  follows: 

1.  Abrasions  of  the  skin. 

2.  Ecchymoses  of  the  skin. 

3.  Wovmds  of  the  soft  parts. 

4.  Fractures  and  injuries  of  the  bones. 

5.  Ruptures  of  internal  organs. 

6.  Tearing  off  entire  portions  of  the  body. 

Abrasions  and  open  wounds  of  the  cutaneous  surface  may  be 
produced  by  the  finger  of  the  examining  physician  or  midwfe;  by 
instruments  or  through  the  pressure  of  the  bony  peh-ic  ring  and  possibly 
by  exostoses  or  tumors  of  the  latter. 

The  pressure  marks  caused  by  projecting  parts  of  the  pehic  rim, 
especially  the  promontory,  are  of  especial  value  to  the  accoucher,  giv- 
ing information  concerning  the  foetal  presentation  and  the  mechanism 
of  labor;  they  form  more  or  less  intensive  areas  of  redness  in  the 
skin,  striated  and  often  containing  haemorrhages.  In  case  the  pressure 
operates  for  a  longer  time  necrosis  of  the  skin  occurs  as  a  result  of  the 
interference  with  its  nutrition.      These  pressure  marks  occur  most  often 

with  vertex  presentations. 

Vol.  II— 1  1 


THE   DISEASES   OF   CHILDREN 


Fig.  1. 


The  application  of  forceps  often  leads  to  circumscribed  compres- 
sions over  the  cranial  and  facial  bones  and  in  consequence  to  suggil- 
lations  and  cede  mas,  excoriations  and  sometimes  necroses  of  the  skin. 

The  wounds  of  the  skin,  covered  with  granulations,  which  are 
occasionally  encountered  in  children  immediately  after  birth  must  be 
ascribed  to  the  tearing  away  of  earUer  formed  adhesions  of  the  skin 
to  amniotic  bands. 

Haemorrhages  occur  frequently  in  unassisted  as  well  as  assisted 
labors.  They  are  in  part  traumatic  in  origin,  in  part,  however,  due  to 
interruption  in  the  placental  circulation  through  compression  of  the 
cord,  or  to  asphyxia.  The  htemorrhages  into  the  skin  are  mostly  only 
punctate    and    very    rarely    assume    larger    proportions.      Among    the 

haemorrhages  of  other  organs 
the  most  frequent  are  ceph- 
alasmatomata,  and  hama- 
tomata  of  the  sternocleido- 
mastoid muscle,  wliich  will 
be  taken  up  in  detail  later; 
also  hajmorrhages  of  the  cra- 
nial and  spinal  meninges  and 
into  the  substance  and  cavi- 
ties of  the  brain  and  cord. 

Meningeal  and  cerebral 
haemorrhages  are  frequent, 
as  a  rule,  giving  rise  to  no 
symptoms;  only  occasionally 
symptoms  of  increased  in- 
tracranial pressure — evident 
cerebral  compres.sion,  slowing 
of  the  pulse,  arrhythmic,  su- 
perficial, intermittent  respirations;  protuberant  or  tense  fontanelle,  con- 
vulsions, and  paralysis — are  observed  immediately  after  birth.  Many  of 
these  children  are  apparently  born  dead,  some  are  normal  at  first  but 
on  the  second  or  third  day  the  breathing  liecomes  shallow  (Kundrat) 
and  the  children  die  with  the  manifestations  of  asphyxia  or  pulmonary 
atelectasis. 

Convulsions. — In  less  acute  cases  attention  is  called  to  the  menin- 
geal bleeding  by  the  occurrence  of  eclamptic  attacks.  The  spasms 
may  be  bilateral  or  unilateral  or  may,  moreover,  be  limited  to  a  single 
extremity,  to  one  half  of  the  face  or  to  the  eyes.  They  may  cease  occur- 
ring after  a  short  time  (just  as  the  paralysis)  or  continuing,  be  the 
indicator  of  a  permanent  disturbance  of  the  cerebral  function. 

We  are  doubtless  justified,  in  occasional  cases,  in  holding  birth 
injuries  responsible  for  permanent  impairment  of  the   brain  function 


^^^^^^^^^^^^^^KVIk^ 

Ps 

:> 

^^^^^^^^^BBb  "v 

^^Hb^'^^ 

^V\^ 

Injury  to  : 


iff   f'l-ur-  Mf  til.'  liprul  as  a  result  of 
nnpn>i>'ii\    ripplicil  forceps. 


DISEASES   OF    THE    NEWBORN  3 

of  the  nature  of  epilepsy,  paralj^ses,  idiocy,  etc.  (Concerning  this 
question  reference  is  made  to  the  chapter  on  Nervous  Diseases.) 

The  diagnosis  of  meningeal  hiemorrhages  in  the  newborn  might 
probably  be  confirmed  l>y  lumbar  ])uneturp:  Finkelstein  was  fortunate 
enough  to  accomplish  this  in  one  case.  The  drawing  off,  on  [nuicture, 
of  a  hu-morrhagic  fluid  does  not  suffice  for  making  a  diagnosis,  as  this 
might  be  due  to  the  puncturing  of  a  vessel.  One  must  find  red  cells 
which  are  altered  morphologically,  possibly  ghosts  or  cell  detritus. 
Kundrat  has  written  expHcitly  concerning  hivmorrhages  localized  in 
the  meninges. 

According  to  Ins  investigations  the  hajmorrhages  are  usuallj'  situ- 
ated under  the  arachnoid  and  in  the  ti.ssues  of  the  pia;  less  frequently 
subdural  hannorrhages  are  found  along  with  these.  Occasionally  intra- 
meningeal  haemorrhages  occur  over  the  cerebellum  and  rarely  into  the 
lateral  ventricles.  As  a  rule  there  are  no  haemorrhages  in  the  substance 
of  the  brain  itself.  According  to  Kundrat,  these  haemorrhages  regu- 
larly occur  in  the  same  manner:  during  the  passage  of  the  fcetus 
there  is  compression  of  the  head,  over-riding  of  the  cranial  bones, 
tension  on  and  tearing  of  the  vessels  or  occlusion  of  the  falci- 
form sinus,  stasis  in  the  veins  emptying  into  this  sinus  and  tearing  of 
these  veins. 

The  ha?morrhages  into  the  lateral  ventricles  are  probably  due  to 
tearing  of  the  vena  magna  Galeni. 

HiPmorrhages  in  other  organs,  such  as  the  muscles,  the  lungs  and 
the  chorioid  membrane  (Sidler-Huquenin)  have  been  often  leported  as 
consequences  of  birth  traumata. 

Fractures  of  the  long  bones  and  the  clavicle  are  most  frequent 
amongst  the  injuries  to  the  bones.  They  are,  moreover,  not  uncommon 
in  unassisted  labor.  Separation  of  the  epiphysis  of  the  humerus  has 
been  found  at  times. 

Dislocations  are  less  common;  that  of  the  shoulder  occasionally, 
and  rarely  of  the  hip-joint. 

Fractures  and  dislocations  of  the  inferior  maxilla  antl  of  tlie  chn'icle 
are  among  the  rarest  of  birth  injuries. 

The  changes  that  occur  in  the  cranial  bones  are  especially  impor- 
tant. The  commonest  result  of  birth  trauma  is  overlapping  of  the  cra- 
nial bones.  The  parietal  bones  lap  over  the  occipital  or  the  frontal  and 
possibly  one  parietal  bone  over  the  other.  This  overlapping  usually 
disappears  within  a  few  days  after  birth.  It  is  brought  about  by  the 
disproportion  between  the  cranial  and  pelvic  diameters;  the  various 
foetal  positions  show  constantly  recurring  types  of  cranial  overlapping. 
Changes  in  the  shape  of  the  cranium,  either  as  a  flattening  or  a  bulg- 
ing, occur  in  many  cases  (according  to  Litzmann,  in  45  per  cent.).  The 
sacral  promontory  especially  exerts  pressure  on  the  contiguous  cranial 


4  THE   DISEASES   OF   CHILDREN 

bones  and  thus  flattens  them  out;  with  this  comes  an  increased  bend- 
ing of  the  bone  opposite,  against  the  symphysis. 

Depressions  of  the  skull  are  serious  occurrences.  One  recognizes 
grooved  and  spoon  (or  funnel)  shaped  depressions;  they  are  usually 
produced  by  pressure  of  the  promontory,  seldom  by  the  symphysis  or 
by  an  exostosis. 

The  flat  pelvis  rather  than  the  generally  contracted  pelvis  produces 
these  indentations;  they  may  be  caused  by  the  pressure  of  forceps 
or,  it  is  said,  by  a  prolapsed  arm  or  leg.  The  deeper  depressions  are, 
as  a  rule,  combined  with  very  shght  fractures  of  the  external  table 
of  the  skull. 

Cephal£ematomata  commonly  occur  at  the  site  of  spoon-shaped 
impressions.  Spoon-shaped  impressions  offer  a  more  unfavorable  prog- 
nosis than  the  gutter-shaped  ones,  often  leading  to  death  and  occasion- 
ally to  cerebral  complications,  as  for  example  convulsions. 

The  attempt  to  reUeve  these  depressions  by  operation  has  been 
made  time  and  again.  Trephining  and  also  elevation  by  means  of 
a  suction  apparatus  have  been  recommended.  Munro  Kerr  suggests 
the  possibihty  of  forcing  out  the  depression  by  compression  antero- 
posteriorly.  ' 

Fractures  of  the  cranial  bones,  lacerations  of  the  sutures  and  tear- 
ing off  of  the  condyloid  processes  of  the  occipital  bone  from  its  tabular 
portion  are  rare  happenings  due  to  the  injudicious  pulling  in  cases  of 
contracted  pelvis. 

In  the  following  sections  the  most  important  birth  injuries  are 
taken  up  in  detail. 

A.     CAPUT     SUCCEDANEUM 
(head-swelling,  breech-swelling) 

Swelhngs  occurring  on  the  presenting  part,  as  the  result  of  com- 
pression,  are  constant   sequelae  of  parturition. 

The  presenting  part,  during  its  passage,  is  tightly  squeezed  by 
the  soft  parts,  especially  by  the  pelvic  diaphragm  (Stumpf)  and  the 
external  os  of  the  uterus;  this  ligature  of  the  presenting  part  causes 
disturbance  in  the  circulation  of  blood  and  lymph,  and  thereby  a  stasis 
arises,  with  outpouring  of  blood  and  serum  into  the  tissues.  The  whole 
of  the  child's  body  except  the  part  in  front  of  the  ligature  is  under  an 
increased  pressure,  possibly  thus  producing  suction  on  the  presenting 
part.  When  the  vertex  presents,  there  is  a  sweUing  of  a  doughy  con- 
sistency, brought  about  by  oedema  of  the  soft  parts,  usually  over  one 
parietal  bone  and  in  fact  usually  over  its  hinder  portion  or  else  over 
the  upper  part  of  the  tabular  portion  of  the  occipital  bone.  This  con- 
sists of  a  sero-hsemorrhagic  infiltration  of  all  the  tissues,  the  greater 
part  of  the  transudation  being  between  the  galea  and  the  periosteum 


DISEASES   OF   THE    NEWBORN  5 

(Lonnberg).  Countless  small  hii'morrhages  are  always  present  in  the 
tissues,  likewise  in  the  periosteum;  at  times  there  is  hypera;inia  of  the 
substance  of  the  bone  and  even  of  the  meninges  beneath  the  swelling. 
In  cases  where  the  labor  is  of  short  duration  the  swellings  are  only 
slightly  developed.  The  factors  determining  the  size  of  a  caput  succc- 
daneuni  are  the  size  of  the  fcrtal  head,  the  dimensions  of  the  maternal 
pelvis  and  the  duration  of  the  hiljor.  A  caput  succedaneum  can  hardly 
be  confused  with  a  cephalajmatoma,  as  the  former  is  not  Umited  by 
the  suture  bones;  it  is  also,  as  a  rule,  more  diffuse  and  pits  on  pressure 
with  the  finger. 

With  breech  presentations  there  exists  a  swelhng  of  the  scrotum 
and  penis,  or,  in  the  female,  of  the  labia  or  nates.  This  swelling  is  also 
not  entirely  made  up  of  serous  effusion  but  contains  many  small  haemor- 
rhages. 

Birth-swellings  occur  in  a  similar  manner  on  the  face  or  on  the 
extremities,  if  these  are  the  presenting  parts. 

B.     CEPHAL^MATOMA 

Hiemorrhage  under  the  galea  aponeurotica  in  the  newborn  was 
given  this  name  by  Naegele. 

Cephaljematomata  occasionally  develop  during  labor,  usually, 
however,  some  time  after  birth  as  a  consequence  of  subperito-osteal 
haemorrhage.  They  usually  develop  between  the  second  and  fourth 
day  of  Ufe,  rarely  as  late  as  the  second  week. 

Cephalsematomata  were  encountered  99  times  in  20,000  births  at 
the  Munich  Lying-in  Hospital  (F.  Beck).  They  have  their  seat  of 
predilection  over  the  parietal  bone,  are  usually  unilateral  and  are 
moreover  on  the  right  side  in  the  majority  of  instances.  They  occur 
at  times  over  both  parietal  bones  or  over  other  cranial  bones,  such  as 
the  frontal,  occipital  and  temporal;  occasionally  several  subperiosteal 
hsematomata  are  found  in  the  same  child. 

On  examination,  a  rounded  mass,  varying  in  its  degree  of  convexity, 
is  .found  over  the  diseased  bone;  the  skin  over  the  tumor  is  movable 
and  either  normal  or,  in  the  first  days  of  hfe,  a^dematous.  At  times 
there  are  haemorrhages  or  pressure  marks. 

During  the  first  days  the  skin  is  usually  tense;  this  tension,  how- 
ever, gradually  diminishes  and  the  tumor  shows  distinct  fluctuation. 
It  occupies  a  larger  or  smaller  part  of  the  parietal  bone  but  never  over- 
steps the  boundaries  set  by  the  sutures  of  the  bone;  inasmuch  as  the  peri- 
osteum is  especially  adherent  at  the  sutures,  the  extravasated  blood  is 
unable  to  separate  the  pericranium  from  the  bone  at  these  points.  The 
swelhng  varies  in  size  from  that  of  a  nut  to  that  of  a  goose  egg  and 
larger.  It  grows  during  the  first  days  after  birth,  attaining  its  maxi- 
mum at  the  end  of  the  first  week. 


6  THE    DISEASES   OF   CHILDREN 

On  palpating  the  tumor,  by  passing  the  finger  over  it  from  periph- 
ery to  dome,  the  impression  of  a  groove  or  defect  in  the  cranium  can 
be  felt.  This  impression  comes  from  the  tumor  being  surrounded  by 
a  hard  wall-hke  ring  of  newly-formed  bony  tissue.  In  its  further  course 
the  tumor  becomes  flatter,  the  wall-like  ring  which  grows  from  the  edge 
towards  the  centre  becomes  broader  until  the  whole  mass  is  covered 
with  a  shell  of  newly-formed  bony  tissue,  more  or  less  dense.  Then  the 
tumor  acquires  a  parchment-hke  sensation,  distinctly  crepitating  on 
pressure  over  the  bony  shell. 

In  other  cases  practically  no  new  growth  of  bone  occurs  or  else  only 
at  the  margin  of  the  effusion;  the  blood  is  then  more  rapidly  absorbed 
and  the  ]ieiiosteum  settles  flush  ^\•ith  the  bone. 

The  formation  of  the  bony  ring  becomes  comprehensible  when  one 
considers  tliat  the  haemorrhage  originates  in  the  torn  vessels  of  the  highly 

Fig.  2.  b 


Schematic  cross-section  through  ceplialrpmatoma  of  about  two  to  three  weeks  duration  :  b.  blood; 
p,  periosteum;  o,  infiltration  with  osteophytes;   k,  bony  tissue;  d,  dura  mater. 

vascular  osteogenetic  tissue  which  lies  next  to  the  connective  tissue 
layer  of  the  periosteum.  The  bleeding  not  only  elevates  the  periosteum 
but  also  a  portion  of  the  osteogenetic  zone.  Hence  arises  the  new 
growth  of  bone  which  is  most  marked  at  the  margin  and  forms  the  wall 
which,  at  times  totally,  at  times  (-nnth  large  effusions)  only  partially, 
covers  the  swelling.  The  periosteal  growth  begins  usually  by  the  end 
of  the  first  week:  up  to  this  time  no  wall  is  palpable.  Tlie  retrogression 
of  the  tumor  follows  sometimes  quickly  and  sometimes  slowly.  The 
absorption  of  the  blood  may  occur  rpiickly  without  the  distinct  forma- 
tion of  new  bone;  in  other  cases  the  absorption  of  the  effusion  and  the 
resolution  of  the  tissues  takes  several  weeks,  usualh'  six  to  eight  and 
sometimes  even  much  longer. 

The  bone  is  either  unchanged  after  the  heaUng  of  a  cephalannatoma 
or  else  presents  a  slight  periosteal  thickening. 

Complications.  — CephaUematomata  do  not  always  progress  so 
smoothly.  Occasionally  the  bloody  contents  of  the  sweUing  become 
infected.     The   infection   usuallv   occurs  from  an   external   wound;  in 


DISEASES    OF    THE    XEWBOHN  7 

rare  cases  its  cause  is  unknown;  sometimes,  however,  it  is  the  result 
of  an  incision  into  the  mass.  Abscess  formation  tiien  occurs,  which  is 
a  serious  matter  because  the  purulent  inflammation  may  extend  to  the 
denuded  bone  or  to  the  soft  parts  of  the  si<in,  whose  movable,  wide- 
meshed  cellular  tissue  furnishes  opportunity  for  the  spreading  of  the  pus 
and  the  extension  of  the  inflammation.  Both  these  comphcations  can 
become  very  dangerous;  the  first  by  causing  an  osteitis  and  extension 
to  the  meninges  and  the  second  by  causing  sepsis. 

Accompanying  cephala-matomata  there  is  found,  very  rarely,  an 
effusion  of  blood  on  the  under  surface  of  the  cranial  bone,  thus  sepa- 
rating the  dura  from  it  {cephalasmatoma  internum).  This  may  occur 
either  with  or  without  a  fracture  of  the  bone;  in  the  latter  case  the 
blood  flows  to  the  under  surface  of  the  bone  through  a  congenital  fis- 
sure. Signs  of  increased  intracranial  pressure  may  then  possibly  arise, 
which,  however,  may  be  caused  in  like  manner  by  a  simultaneous  cere- 
bral or  meningeal  ha-morrhage. 

Pathology. — In  children  dying  immediately  after  birth,  the  skin 
over  the  cephala-matoma  is  cedematous  and  richly  besprinkled  with 
hsemorrhages.  The  periosteum  is  elevated,  darkly  discolored  and  also 
full  of  small  htemorrhages;  between  it  and  the  bare,  rough  bone  the 
dark  fluid  is  gathered  and  a  few  clots  of  fibrin  cUng  to  the  walls. 

In  cases  in  which  the  child  dies  some  time  after  birth,  there  are 
signs  of  a  periosteal  growth  of  new  bone  either  at  the  margin  of  the 
effusion,  or  later,  also  on  the  inner  surface  of  the  whole  roof  of  the 
tumor;  this  is  soft  at  first,  offering  no  resistance  to  the  knife;  but  later 
it  becomes  hard  and  then  colloid  masses  or  irregular  lamelhe  of  new 
bone  are  found  over  the  bone. 

With  cephalcEmatoma  internum  similar  anatomical  changes  are 
found  with  the  addition  of  the  signs  of  a  fracture  or  else  evidence  to 
show  that  the  blood  has  trickled  through  a  pre-existing  fissure.  In  some 
cases  there  are  also  cerebral  hemorrhages.  With  complications,  condi- 
tions are  encountered,  often  extensive,  corresponding  to  the  chnical 
picture. 

Pathogenesis. —  Cephala>matomata  are  caused  by  ;i  tearing  of 
the  vessels  of  the  subperiosteal  zone,  with  the  pouring  forth  of  l)lood 
and  the  resultant  elevation  of  the  periosteum.  The  bursting  of  a  vessel 
is  as  a  rule,  caused  by  stasis  and  hypersemia  (M.  Runge). 

Because  the  vessels  are  easily  torn  and  the  periosteum  is  loosely 
connected  with  the  bones  of  the  skull  in  the  newborn,  stasis  readily 
leads  to  the  formation  of  a  cephahpmatoma.  This  simple  explanation 
of  Runge's  makes  it  easy  to  understand  just  why  the  parietal  bone  and 
especially  the  right  parietal  bone  should  so  often  be  the  seat  of  cephal- 
spmatoma.  This  is  due  to  the  preponderance  of  L.  0.  A.  positions, 
with   which   the  right    parietal   bone   presents,   so   that  stasis  and    the 


8  THE   DISEASES   OF   CHILDREN 

bursting  of  vessels  take  place  oftenest  over  it.  Whether,  in  this  event, 
only  small  haemorrhages  or  a  cephalsematoma  ensues  depends  princi- 
pally upon  the  size  of  the  ruptured  vessels. 

Another  theory  (Fritsch)  states  that  stasis  is  not  so  much  responsi- 
ble for  the  occurrence  of  a  cephala'matoma  as  is  the  mechanical  loosen- 
ing of  the  periosteum  by  means  of  a  trauma,  such  as  may  occur,  more- 
over, during  the  course  of  nornuil  lalior.  The  f(Ttal  head  becomes  fixed 
in  the  birth  canal  and  the  scalp  sticks  tightly  to  the  maternal  soft  parts; 
in  the  interim  between  pains  the  head  recedes,  thus  pulling  on  the  scalp 
and  separating  the  periosteum  from  the  cranium. 

Other  authors  assume  only  a  localized  pressure  operating  on  the 
cranium  with  a  resultant  rupture  of  blood  vessels.  Runge's  explana- 
tion is  the  most  plaasible;  but  it  must  be  conceded  that  the  assumption 
of  a  pressure  operating  locally,  makes  the  explanation  more  easily  under- 
standable. Otherwise  it  would  not  be  possible  to  explain  the  occur- 
rence of  cephahx-matoma  in  places  where  the  blades  of  forceps  have 
pressed  on  the  head.  The  cases,  happily  rare,  where  a  fracture  has  been 
followed  by  a  cephala?matoma  also  argue  for  this.  In  the  vast  ma- 
jority of  instances,  however,  cephaltematomata  occur  in  uneventful, 
uncomplicated  labors. 

From  a  large  number  of  observations  concerning  the  circumstances 
under  which  cephalsematomata  occur  the  follo^dng  conclusions  are 
drawn  by  F.  Beck. 

Cephalsematomata  occur  most  frequently  over  the  right  parietal 
bone;  four-fifths  of  all  cases  occur  in  primiparse;  primiparse  constitute 
about  one-half  of  all  births.  The  rigidity  of  the  maternal  soft  parts 
has,  therefore,  a  considerable  influence;  this  is  also  shown  by  the  fact 
that  the  cephalgematomata  occur  more  frequently  in  the  children  of 
elderly  primiparse  than  those  of  j'ounger  mothers.  Slightly  contracted 
maternal  pelves,  abnormal  foetal  positions  and  premature  rupture  of 
the  amniotic  sac  are  of  importance  since  thereby  the  head  may  be 
easily  subjected  to  irregularly  or  sporadically  apphed  pressure. 

The  infants  in  whom  cephaUfmatomata  occurred  were,  for  the 
most  part,  full-term  children,  but  premature  infants  are  not  immune. 
It  is  a  striking  fact  that  all  statistics  show  a  substantial  preponderance 
of  male  over  female  infants  (about  two  to  one);  this  probably  depends 
on  the  larger  head  measurements  in  male  children.  A  considerable 
number  of  the  children  are  born  asphyctic;  aspltyxia  conduces  to  the 
formation  of  cephalsematoma  by  causing  increased  hypersemia  and  also 
because  of  the  fact  that  under  its  influence  the  nutrition  of  the  vessel 
walls  suffers. 

The  diagnosis  is  in  general  easily  estabUshed.  Only  in  the  first 
days,  a  caput  succedaneum,  which  often  accompanies  a  cephala^ma- 
toma,  may  possibly  cause  doubt.     It  is  easily  differentiated  from  trau- 


DISEASES    OF    THE    XEWBOHX  9 

matic  meningocele  since  the  latter  swells  when  the  child  cries  and 
can  be  emptied  by  pressure.  A  cephakematoma  subajjoneuroticum,  a 
ksemorrhage  between  the  scalp  and  the  periosteum,  is  easily  differen- 
tiated from  the  subperiosteal  form  by  the  fact  that  it  is  not  limited  by 
the  suture  lines,  which  it  oversteps. 

The  prognosis  is  good.    Complications  are  rare. 

The  therapy  as  a  rule  should  be  conservative.  Protection  from 
presi5ure  by  means  of  heavy  cotton  padding  fixed  l)y  means  of  a  hood, 
almost  always  insures  a  nice  hcahng  of  the  swelling.  Iiu-i.sion  is  to  be 
considered  only  in  the  cases  where  the  tumor  is  very  large;  and  here, 
under  the  strictest  asepsis,  it  is  to  be  preferred  to  all  other  procedures. 
The  majority  of  ph3'sicians,  however,  rightly  oppose  operative  inter- 
ference since  thereby  the  danger  of  infection  is  substantially  increased. 
Incision  must  be  employed,  however,  in  every  case  where  the  skin  over 
the  sweUing  becomes  red  and  adematoua:  for  then  there  is  suppuration 
of  the  effusion  and  only  timely  and  thorough  opening  and  dressing  with 
dermatol  gauze  or  other  antiseptics  can  halt  the  spread  of  the  inflamma- 
tion. The  employment  of  puncture  for  the  purpose  of  emptying  the 
blood  and  the  use  of  compression  bandages  are  not  recommended;  it 
is  very  difficult  to  applj'  bandages  which  really  exert  compression  and 
this  procedure  is  apt  to  injure  the  scalp. 

C.     ILEMATOMA   AND   MYOSITIS   OF   THE   STERNO- 
CLEIDOMASTOID   MUSCLE 

Dieffenbach  first  described  this  condition  in  1830  and  also  first 
brought  out  its  causal  relation  with  congenital  wry  neck. 

Symptoms. — Soon  after  birth,  or  a  few  days  later,  a  tumor,  of  a 
hard,  cartilage-like  consistency,  is  found  in  the  sternocleidomastoid 
muscle  and  moreover  usually  in  its  sternal  portion;  the  tumor  varies 
in  size  from  that  of  a  hazel-nut  to  that  of  a  jjigeon  egg  and  over  it  the 
skin  is  usually  unchanged  although  at  times  cedematous.  When  the 
muscles  are  relaxed  the  tumor  is  easily  movable  mth  the  sternomas- 
toid;  it  is  not  painful,  although  at  times  tender  on  pressure.  In  some 
instances  the  chin  is  pointed  toward  the  healthy  side,  in  some  toward 
the  diseased  side  and  in  others  the  positon  of  the  head  is  normal.  The 
general  health  is  not  disturbed. 

Nature  and  Pathological  Anatomy. — In  this  condition  we  have 
to  deal  with  a  tearing  of  the  fibres  of  the  sternomastoid  with  a  conse- 
quent bleeding.  The  blood  flows  under  the  muscle-sheath  and  between 
the  torn  fibres;  in  cases  of  considerable  duration  it  is  possible  to  demon- 
strate, microscopically,  the  partial  disappearance  of  the  muscle  fibres 
and  proliferation  of  young  connective  tissue. 

Occurrence  and  Pathogenesis. — The  disease,  in  the  majority  of 
instances,  occurs  in  children  dehvered  without  the  aid  of  instruments. 


10  THE    DISEASES   OF   CHILDREN 

The  right  side  is  more  often  affected  than  the  left,  in  rare  instances 
both  sternomastoids  are  affected.  The  disease  occurs  most  often  with 
breech  presentations,  less  often  with  vertex  presentations.  In  cases 
of  unassisted  labor  the  occurrence  of  the  hiematoma  must  be  attributed 
to  an  extreme  traction  on  the  stcrnomastoid;  this  traction  can  take 
place  only  from  an  excessive  rotation  of  the  head  (Kiistner)  and  more- 
over the  stcrnomastoid  which  remains  inactive  during  the  act  of  rota- 
tion is  subjected  to  the  greater  traction;  therefore  the  muscle  affected 
corresponds  to  the  side  towards  which  the  head  is  rotated.  In  a.ssisted 
labor  also,  rotation  of  the  head  is  responsible  for  the  tearing  of  the 
muscle  although  pressure  of  the  forceps,  of  the  fingers  of  the  accoucheur 
or  of  the  cord  wound  round  the  neck  are  said  to  be  able  to  ])roduce 
this  lesion. 

The  prognosis  is  good.  The  tumor  usually  disappears  spontaneously 
within  a  few  (4-8)  weeks.  Proof  is  wanting  that  a  permanent  wry  neck 
ever  develops  from  this  condition. 

The  treatment  consists  in  massage  of  the  diseased  muscle,  carried 
out  very  gently,  for  several  minutes  daily.  In  case  the  disease  has 
existed  for  some  time  and  wry  neck  has  developed,  it  is  recommended 
to  employ  passive  movements  of  the  head,  in  the  sense  of  rotation  away 
from  the  affected  side. 

D.    OBSTETRICAL   PARALYSES 

From  the  large  category  of  paralyses  caused  by  injury  to  the  new- 
born during  parturition,  there  are  classified  under  the  title  obstetrical 
paralyses,  only  those  confined  to  the  upper  extremity  and  due  to  injury 
of  the  brachial  plexus.  Its  symptom-complex  is  identical  with  plexus 
paralysis  in  the  adult  and  it  deserves  special  consideration  only  on 
account  of  its  etiology  and  the  regularity  of  the  nervous  symptoms 
following  the  birth-trauma.  Duchenne  accurately  described  the  disease 
and  Erb  elucidated  its  nature.  Subsequent  authors  have  only  substan- 
tiated the  results  of  the  studies  and  observations  of  the  above  named 
and  have  broadened  and  deepened  our  knowledge  of  the  clinical  mani- 
festations and  pathogenesis  of  the  disease. 

Symptoms. — Inactivity  is  noticed  in  the  diseased  arm  immediately 
after  birth.  AVhereas  the  healthy  extremity,  especiahy  shortly  after 
birth,  shows  a  rather  marked  rigidity  of  its  musculature  and  whereas 
the  child  executes  various  more  or  less  extensive  movements,  with  it, 
the  diseased  arm  hangs  relaxed,  and  it  is  impossible,  by  pricking  the 
skin  to  elicit  active  lifting  of  the  arm  at  the  shoulder  or  flexion  of  the 
elbow.  The  shoulder  hangs  somewhat  lower  than  the  unaffected  one; 
this  is  less  marked  in  very  recent  cases  than  in  older  ones.  The  upper 
arm  is  rotated  inwards  and  the  forearm  pronated  so  that  the  palm  of  the 
hand  is  turned  more  or  less  outwards.     The  movements  of  the  scapula 


DISEASES  OF  THE   NEWBORN 


11 


are  either  not  interfered  with  at  all  or  else  only  slightly.  Supination 
in  the  elbow-joint  is  always  absent;  the  wrist-joint  seems  either  free 
from  involvement  or  else  extension  is  limited,  flexion  remaining  possible. 
The  finger-joints  as  a  rule  are  free  from  disturbances  although  rarely 
they  suffer  limitation  in  flexion.  The  sensibihty  is  either  undisturbed 
or  there  may  be  disturbances  of  sensation  in  the  distribution  of  the 
musculocutaneous  and  more  rarely  the  axillary  nerve  (Oppeidieim); 
even  with  very  extensive  paralyses,  the  sensibihty  on  the  inner  surface 
of  the  arm  remains  normal  (Klumpke).  Tests  of  the  electrical  reactions 
in  the  newborn  give,  in  general,  results  of  but  little  practical  value, 
since  the  irritabihty  is  physiologically  less  as  compared  with  older  chil- 
dren and  adults,  and  the  contractions  themselves,  physiologically, 
tardy  and  vermiform  (C.  Soltmann  and  A.  Westphal).  The  reaction  of 
degeneration  and  other  similar  reactions  are  not  to  be  employed  in  the 


Fig.  3. 


VIII 


N.  dorsalis  I 


N.  thoraicu? 
longus 


Brancli  to  M.  supraspiii. 
infraspiii.  teres  minor 


N.  subscapulari.s 


N.  inu>ciilocutaneus 


N.  medianus 


N.  ulnaris  . 


N.  radialis 


Brachial  plexus. 

same  sense  as  in  the  adult.  The  form  of  the  disease  described  as  the 
type,  by  Duchenne  and  Erb,  is  the  most  frequent,  occurring  according 
to  .Stransky  in  80  per  cent,  of  all  cases.  The  disease  depends  upon  a  pe- 
cuUar  combination  of  muscle-palsies  which  is  manifest  in  all  these  cases 
and  the  rationale  of  the  occurrence  of  which  was  rightly  recognized  by 
Erb.  The  following  muscles  are  involved:  deltoid  (hfter  of  the  arm), 
biceps  (flexor  of  the  forearm),  infraspinatus  (external  rotator  of  arm) 
supinator  longus  (flexor,  possibly  supinator  of  the  forearm)  and  supi- 
nator brevis  (supinator  of  the  forearm).  These  muscles  are  supplied 
by  the  brachial  plexus  which  is  formed  by  branches  of  the  four  lower 
cervical  and  the  greater  part  of  the  first  dorsal  nerves.  Erb  found  a 
point  corresponding  to  the  point  where  the  sixth  cervical  nerve  emerges 
between  the  muscuh  scaleni,  from  which  point  it  is  possible  with  the 
faradic  current,  to  produce,  simultaneously,  contractions  in  the  above- 


12  THE   DISEASES   OF   CHILDREN 

named  muscles  (deltoid,  biceps,  brachialis  anticus,  supinator  longus 
and  supinator  brevis).  A  lesion  at  this  point  produces  the  above- 
described  symptom-complex. 

Whereas  the  Erb-Duchenne  type  involves  muscles  supplied  by  the 
fifth  and  sixth  cervical  nerves  {upper  plexus  paralysis,  upper  arm  type), 
there  is  another  rarer  form  of  obstetrical  paralysis  in  which  muscles 
supplied  by  the  seventh  and  eighth  cervical  nerves  are  involved  either 
alone  or  together  with  the  muscles  affected  in  the  upper  plexus  paral- 
ysis {lower  plexus  paralysis,  Klumpke's  type,  lower  arm  type).  In  such 
cases  we  have  a  fairly  complete  paralysis  of  the  arm,  forearm  and 
fingers,  with  extensive  sensory  involvement  and — especially  character- 
istic— oculopupillary  symptoms:  narrowing  of  the  palpebral  aperture, 
and  a  myosis  in  which  the  contracted  pupil  promptly  reacts  to  Ught  and 
accommodation.  The  narrowing  of  the  palpebral  aperture  is  caused 
by  a  ptosis  which,  as  well  as  myosis,  is  due  to  an  involvement  of  the 
sympathetic  nerve  (Seeligmiiller),  the  communicating  branch  of  which, 
coming  from  the  first  dorsal  nerve,  connects  with  the  lowest  part  of  the 
brachial  plexus  and  is  involved  in  the  lesion.  Seeligmiiller  also  reports 
a  case  in  which  there  was  an  atrophy  of  the  cheek.  Klumpke's  type  is 
to  be  met  with,  uncompUcated,  in  only  a  part  of  the  cases,  in  the  others, 
the  muscles  of  the  Erb-Duchenne  type  are  also  involved  and  in  occa- 
sional cases  all  the  muscles  of  the  upper  extremity  are  paralyzed. 

The  upper  plexus  paralysis  however  does  not  always  extend  to 
all  of  the  above-named  muscles;  in  rare  cases,  muscles  are  affected 
singly,  as,  for  example,  isolated  deltoid  paralysis;  in  other  children,  be- 
sides the  muscles  of  the  Erb-Duchenne  type,  additional  muscles,  e.g., 
subscapularis  rhomboideus,  serratus  and  pectorahs  major  may  be  con- 
comitantly involved.  Bilateral  plexus  paralysis  has  been  observed 
very  rarely. 

In  the  course  of  the  following  months,  atrophies  and  contractures 
occur  in  the  cases  which  do  not  recover;  prominence  of  the  shoulder 
bones  and  lateral  flattening  of  the  shoulder  are  characteristic  for  a  paral- 
ysis of  considerable  duration.  The  bony  growth  of  the  affected  extrem- 
ity is  retarded  while  the  general  development  of  the  child  advances;  a 
subluxation  of  the  humerus  in  the  shoulder-joint  sometimes  follows, 
which  of  itself  materially  hmits  the  usefulness  of  the  extremity.  The 
most  important  compUcations  of  obstetrical  paralysis  are  injuries  to 
the  bones  and  joints  which  modify  the  clinical  picture  and  which  may 
even  relegate  the  symptoms  of  the  paralysis  into  the  background.  Frac- 
tures of  the  humerus,  clavicle  and  scapula,  and  epiphyseal  separation 
at  the  upper  end  of  the  humerus  are  not  uncommon.  Facial  palsy  and 
wry  neck  often  accompany  the  paralysis.  A  predisposing  role  for  the 
occurrence  of  obstetrical  paralysis  has  even  been  ascribed  to  wry  neck 
(Schiiller).     The  few  anatomical  findings  of  obstetrical  paralysis  show. 


DISEASES   OF   THK    NEWBORN  13 

in  recent  cases  an  extravasation  of  blood  into  the  plexus  or  else  a  tear- 
ing of  its  fibres;  in  a  case  of  longer  duration  (examined  by  Oppcnheini 
and  Nonne)  degenerations  were  found  in  the  distribution  of  the  fifth  and 
sixth  cervical  roots. 

The  etiology  of  obstetrical  paralysis  is  still  somewhat  obscure. 
Whereas  Erb  holds  energetic  pressure  of  the  fingers  over  the  plexus 
(especially  in  the  apphcation  of  the  Prague  mana'uvre)  responsible,  the 
present  opinion  is  that  pressure,  either  of  forceps,  or  of  a  narrow  pelvis, 
of  a  clavicle  or  finger  in  carrying  out  the  method  of  Mauriceau,  or 
else  the  tearing  and  stretching  resulting  from  wrongly  directed  traction 
with  forceps,  especially  with  excessive  flexion  of  the  head  or  bending 
of  the  head  during  the  evolving  of  the  .shoulder,  may  be  each  at  times 
responsible  for  obstetrical  paralysis.  Under  these  manipulations  the 
fifth  and  sixth  cervical  nerves  would  suffer  principally  (Fieux  et  ai). 
According  to  Peters  the  pure  tj^pe  of  Duchenne-Erb's  palsy  occurs  only 
in  children  born  with  the  breech  presenting.  Obstetrical  paral3'sis  has 
only  been  Uxice  observed  in  unassisted  labor.  Stransky  calls  attention 
to  asphyxia  and  the  resultant  hypervenosity  of  the  blood  as  a  favoring 
element  through  which  the  peripheral  nerves  are  rendered  more  sus- 
ceptible to  trauma. 

The  diagnosis  is  easily  established.  Errors  may  be  made  in  differ- 
entiating the  condition  from  an  immobility  of  the  extremity  due  to 
enlargements  of  the  bones.  A  separation  of  the  upper  epiphysis  of  the 
humerus,  which  hke^\ise  is  accompanied  by  inward  rotation  of  the  arm, 
is  said  to  simulate  plexus  paralysis  rather  freciuently  (Kiistner).  Care- 
ful examination  and  the  use  of  the  Rontgen  rays  will  protect  against 
this  error.  The  possibiUty  of  congenital  syphilitic  pseudoparalysis  or  of 
congenital  peripheral  paralysis  must  be  considered  and  excluded  ])y 
careful  scrutiny  of  the  symptom-complex.  Infantile  cerebral  paralysis 
will  hardly  cause  confusion  in  diagnosis  because  of  the  different  state 
of  the  muscular  tone  of  the  reflexes  and  the  distribution  of  the  paralj'ses. 

The  prognosis  varies:  the  fewer  the  number  of  muscles  involved, 
the  quicker  the  prompt  irritabihty  is  re-establi.shed  and  the  sooner  the 
treatment  is  instituted,  the  better  the  prognosis.  The  majority  of 
obstetrical  paralyses  recover  fully:  however  a  considerable  number 
resist  treatment  either  entirely  or  in  part. 

The  therapy  consists  in  the  early  apphcation  of  the  faradic  or  gal- 
vanic current  to  the  diseased  muscle;  the  treatment  should  be  carried 
out  for  several  minutes  daily.  Massage  and  passive  movements  are  used 
to  combat  the  occurrence  of  atrophies  and  contractures.  Splints  and 
other  orthopedic  appHances  are  employed  in  older  cases;  plastic  opera- 
tions on  the  tendons  and  nerves  come  into  consideration.  Mikuhcz  ob- 
tained a  good  result  in  a  baby  five  weeks  old,  by  stretching  the  plexus 
which  he  had  exposed  with  the  knife. 


14  THE   DISEASES   OF   CHILDREN 

APPENDIX 

Besides  the  typical  obstetrical  paralysis  of  the  upper  extremity, 
injuries  to  other  nerves  occur,  most  frequent  among  which  is  a  periph- 
eral facial  paralysis.  This  may  be  uni-  or  bilateral;  all  or  only  one  of 
the  branches  of  the  facial  nerve  may  be  involved.  The  paralysis  occurs 
as  a  rule  in  instrumental  labor  as  a  result  of  the  pressure  of  the  blade  of 
the  forceps  on  the  trunk  of  the  facial  nerve.  It  occurs  only  very  excep- 
tionally in  unassisted  labor.  It  may  be  caused  by  an  cedema  or  a  haema- 
toma  in  the  trunk  of  the  facial  nerve,  which  has  been  produced  by  the 
pressure  of  the  bony  pelvic  ring,  in  cases  of  contracted  pelvis. 

The  diagnosis  is  made  as  in  facial  paralysis  from  other  causes 
(which  see);  the  possibility  of  the  paralysis  being  central  [e.g.  from 
congenital  cerebral  paralysis  or  congenital  mal-development  of  the 
facial  muscles)  must  be  considered  in  every  case.  The  prognosis  is  rela- 
tively a  good  one.  The  paralysis  disappears  in  the  most  instances 
within  a  few  months. 

Obstetrical  paralyses  of  the  lower  extremities  have  not  often  been 
observed.  Injuries  and  tears  of  the  spinal  cord  occur  only  with  exces- 
sively energetic  attempts  at  extraction  and  as  a  rule  lead  to  the  death 
of  the  child.  Htemorrhages  into  the  spinal  cord,  with  rare  exceptions, 
give  rise  to  no  clinical  symptoms. 

II.     DISTURBANCES  RESULTING  FROM  THE  CHANGE 
TO  EXTRA-UTERINE  LIFE 

A.    ALBUMINURIA  AND  URIC  ACID  INFARCTION  OF  THE  NEWBORN 

In  the  first  days  of  its  life  the  infant  passes  but  little  urine;  the 
scanty  ingestion  of  fluids  and  the  active  loss  of  water  through  the  skin 
and  lungs  reduce  the  kidney-secretion  to  a  level  lower  than  that  cor- 
responding to  the  bod}^  weight.  Regular  examinations  of  the  urine 
show  the  striking  fact  that  albumin  is  present  for  one  or  more  days  in 
the  urine  of  at  least  one  half  of  all  newborn  infants  and  according  to 
the  latest  investigations  in  all.  This  albumin  is  nucleo-albumin;  more 
rarely  some  other  proteid  suljstance  (Flensburg).  The  assumption  that 
nmcin  is  present  in  the  urine  of  newborn  children  (Cruse)  has  not  been 
substantiated.  The  amount  of  albumin  varies  but  is  never  large.  The 
excretion  of  albumin  begins  after  birth  (urine  found  in  the  bladder  at 
birth  is  usually  free  from  albumin),  lasts  rarely  only  one,  usually  the 
first  four  days  and  is,  as  a  rule,  ended  within  the  first  nine  days  of  life 
(Cruse).  In  very  rare  cases  it  has  been  known  to  persist  as  long  as  the 
second  month  of  Ufe. 

The  cause  of  this  albuminuria  has  not  been  definitely  settled.  It 
possibly  has  a  connection  with  infarction  of  the  kidney  in  the  newborn 
(Hof meister) ;  at  least  it  is  true  that  the  height  of  the  infarct  formation 
is  often  accompanied  by  considerable  albuminuria   (Flensburg).     The 


DISEASES    OF   THE    NEWBORN  15 

absence  of  urates  does  not  argue  against  this,  since  infarctions  may  be 
present  in  the  kidney  without  there  l)eing  anything  ilemonstraljlc  in  the 
urine.  Witli  tliis  assumption  as  a  basis  one  could  attril)ute  I  lie  nucleo- 
albumin  of  the  newborn  to  some  damage  to  the  [larenchyma  of  the  kid- 
ney by  uric  acid  infarction.  Nucleo-albumin  does  not  come  from  the 
blood,  since  none  is  present  there;  it  has  its  origin  in  the  cells  of  the 
parenchyma  of  the  kidney.  The  question,  whether  albuminuria  in  the 
newborn  is  a  physiological  process  which,  according  to  Virchow,  depends 
on  revolutionary  changes  in  the  metabolism  of  the  newborn  at  the 
moment  of  birth,  remains,  according  to  Czerny  and  Keller,  as  yet 
undecided. 

The  urine  of  the  newborn  is  clear,  immediately  after  birth;  later, 
however,  it  usually  is  cloudy  and  remains  so  for  4-5  days.  Microscopi- 
cally the  urinary  sediment  shows  the  presence  of  pavement  epithehum 
from  the  peripheral  urinary  j^assages,  also  leucocytes,  hyaline  anfl  epi- 
theUal  casts,  renal  epithehum  and  amorphous  hyaline  substance  (Cruse, 
Flensburg).  According  to  Reusing,  casts  are  present  in  the  urine  of 
39.4  per  cent,  of  breast-fed  children  and  only  9.1  per  cent,  of  artificially 
fed  children.  This  is  connected  with  the  larger  ingestion  of  fluids  by 
the  children  fed  on  cow's  milk,  who  thus  secrete  a  less  concentrated 
urine  which  is  less  damaging  or  irritating  to  the  kidney  epithehum. 
Finally  a  brick-red  sediment  appears  in  the  urine,  often  even  on  the 
first  day,  but  usually  on  the  second  to  the  fourth,  which  according  to 
Flensburg  has  been  analyzed  by  Sjoqvist  and  found  to  be  composed  of 
urate  of  ammonium;  with  a  hyahne  substance  for  a  nucleus  it  gathers 
in  rods  or  balls  and  often  contains  incrusted  epithehal  cells  or  casts. 
This  sediment  owes  its  origin  to  the  uric  acid  infarctions  of  the  neicborn. 
It  is  only  excreted  during  the  first  days  of  life,  usually  from  the  second 
to  the  fourth  day  and  rarely  later  than  the  beginning  of  the  second 
week.  According  to  Flensburg  these  infarcts  are  present  in  all  newborn 
children.  Where  they  are  present  in  considerable  proportions  brick- 
red  spots  are  seen  on  the  diaper  of  the  child;  retention  of  urine 
in  the  newborn  seems  sometimes  to  be  connected  with  the  ehmination 
of  infarctions.  In  this  case  the  first  urine  is  voided  mthout  diffi- 
culty, but  later  retention  and  restlessness  set  in  and  may  persist  for 
many  hours. 

Uric  acid  infarctions  are  rarely  found  in  the  kidneys  of  still-born 
infants.  In  children  who  have  lived  for  a  time,  however  (hours,  days  or 
weeks),  one  exceptionally  often  finds  that  the  pyramids  alone  of  the 
kidney  are  striped  with  a  large  number  of  reddish-  or  brownish-yellow, 
often  also  hght  yellow  fines;  under  the  microscope  these  turn  out  to  be 
balls,  granules  or  rods  of  uric  acid  salts  embedded  in  an  organic,  pro- 
teid-hke  substance.  A'irchow  con.sidered  the  urate  to  be  the  ammonium 
salt.     The  occurrence  of  these  infarcts  has  not  yet  been  explained.     It 


16  THE   DISEASES   OF   CHILDREN 

is  true  that  Reusing,  Sjoquist  and  others  have  estabhshed  the  very  high 
percentage  of  uric  acid  in  the  urine  of  the  newborn;  since  however,  in 
adults  with  very  high  percentages  of  uric  acid,  e.g.  in  cases  of  leukaemia, 
infarctions  have  never  been  described;  it  becomes  necessary  to  assume 
other  pecuhar  relations  in  order  to  explain  the  infarct  formation.  Flens- 
burg  believes  that  a  proteid-Uke  substance  is  secreted  in  fcetal  Ufe  and 
during  the  first  days  after  birth,  which  gathers  in  the  convoluted  tu- 
bules, interferes  with  the  passing  off  of  the  urine  and  becomes  incrusted 
from  the  passage  of  the  urine  rich  in  uric  acid.  Spiegelberg  was  able 
to  show  that  uric  acid  infarctions  follow  the  injection  of  urates  in  new- 
born animals  but  never  in  adults.  The  explanation  of  this  was  not 
forthcoming  from  his  researches. 

B.  PREGNANCY-REACTIONS   IN   THE    NEWBORN    AND   THE 
DISTURBANCES   ARISING    THEREFROM 

After  birth,  in  the  body  of  the  infant  certain  peculiar  conditions 
are  noticeable  which  we  designate,  with  J.  Halban,  "pregnancy-reac- 
tions," since  thej'  are  connected  with  the  circulation  of  certain  bodies 
in  the  blood  of  the  pregnant  woman  and  with  the  carrying  of  these  sub- 
stances over  into  the  blood  of  the  foetus.  Hence  we  find  changes  in  the 
newborn  which  entirely  disappear  within  the  first  weeks  of  life,  never 
to  recur  in  the  male,  and  in  the  female  only  during  puberty  and 
pregnancy. 

I.    SECRETION    OF    THE    MAMMARY  GLAND   IN    THE    NEWBORN 

Symptoms. — On  about  the  second  or  third  day,  rarely  later,  one 
notices  almost  invariably  in  every  newborn  child,  without  regard  to 
sex,  a  swelhng  of  the  breast  which  increases  on  the  following  days  and 
usually  reaches  its  acme  from  the  eighth  to  the  twelfth  day.  From  this 
time  on  the  swelhng  gradually  diminishes  and  disappears  completely  in 
the  third  or  fourth  week.  The  skin  is  entirely  unchanged  over  the  swell- 
ing. On  squeezing  the  gland  a  milky  secretion  exudes  which  is  called 
"witch's  milk."  This  secretion  has  been  examined  repeatedly  and  con- 
tains much  albumin,  casein,  fat,  milk-sugar,  and  salts;  its  ash  contains 
chlorine,  phosphoric  acid,  sodium,  potassium,  magnesium  and  iron. 
It  is  similar  in  composition  to  colostrum.  Chemical  analysis  of  this 
secretion  in  Genser's  case  gave  the  following  quantities  per  htre:  5.57  Gm. 
casein;  4.90  Gm.  albumin;  9.56  Gm.  milk-sugar;  14.56  Gm.  fat;  8.26  Gm. 
inorganic  salts.  Microscopically  are  found  milk  globules,  leucocytes  and 
so-called  colostrum  corpuscles  which,  according  to  Czerny,  are  milk  glob- 
ules laden  with  leucocytes,  or  according  to  others  with  epithehal  cells. 
The  secretion  of  milk  persists  usually  into  the  11th  month.  It  is  said  that 
this  secretion  may  be  kept  up  for  a  very  long  time  by  regularly  emptying 
the  gland  of  its  contents.  This  secretion  of  the  mammary  gland  in  the 
newborn  must  be  regarded  as  a  physiological  process.     Whereas  former 


DISEASES    or   THE    NEWHORN  17 

theories  sought  to  explain  this  secretion  on  the  ground  of  a  fatty  meta- 
morphosis of  the  central  cells  of  the  fcetal  "anlage"  of  the  gland,  which 
is  solid  (Kolhker),  or  with  a  desquamation  of  the  glandular  epithelium 
(Epstein),  we  at  present  beheve  that  the  same  stimulation  which  calls 
forth  the  development  of  the  mammary  gland  in  the  mother  operates 
on  the  mammary  gland  of  the  fcrtus  and  produces  the  same  reaction 
(Knopfelmacher).  According  to  J.  Halban  this  agent  is  a  secretion  of 
the  placenta  and  moreover  of  the  epithehum  of  the  chorion.  The  secre- 
tion passes  into  the  maternal  blood  and  then  into  the  fcetal  circulation 
and  causes  the  development  of  the  lacteal  gland.  This  explanation  how- 
ever does  not  cover  the  fact  that  the  secretion  of  milk  does  not  start 
until  the  fa^tus  has  been  born.  At  birth  the  mammary  gland  of  the 
newborn  shows  similar  changes  to  that  of  the  mother;  proUferated, 
feebly  staining  epithelium,  dilated  ducts,  and  surrounding  the  ducts, 
haemorrhages,  leucocytes,  eosinophiles  and  giant  cells.  Shortly  after 
birth  the  so-called  "puerperal  involution"  (Halban)  commences,  which 
operates  upon  the  breast  of  the  child  in  the  same  way  as  it  does  upon 
that  of  a  non-nursing  mother.  The  excitation  of  the  milk  secretion  after 
birth,  which  jirobablv  depends  upon  the  same  cause  in  both  mother  and 
child,  is  supposed  to  be  due  to  a  cessation  of  the  placental  action.  A 
ferment  secreted  by  the  placenta  inhibits  the  secretion;  its  cessation, 
as  the  result  of  the  birth,  is  followed  by  secretion.  Scliein  assumes  a 
hypera-mia  of  the  gland  to  be  responsible  for  this.  This  explanation, 
however,  does  not  seem  to  be  satisfactory  and  we  must  say  that  the  cause 
for  the  excitation  of  the  milk  secretion  is  not  yet  clearlj'  established. 

II.    M.\STITIS    IX    THE    NEWBORN 

Inflammation  of  the  mammary  gland  nearly  always  occurs  in  a 
functionating  organ.  Hence,  acute  inflammations  of  the  breast  some- 
times occur  in  the  newborn  just  as  in  women  in  the  puerperium  and 
during  lactation.  In  the  child  the  disease  occurs  only  from  the  1st  to  the 
3rd  week  of  hfe.  At  the  beginning  of  the  attack  the  breast  becomes 
tender  and  gradually  becomes  reddened,  and  the  skin  over  it  cedema- 
tous.  The  vicinit}'  of  the  gland  protrudes  as  a  whole  and  gradually 
suppurative  softening  takes  place  followed  by  spontaneous  rupture, 
unless  opened  surgically.  The  disease  is  often  accompanied  by  very 
high  fever,  restlessness,  anorexia,  vomiting  and  hquid  stools.  Mastitis 
must  be  attributed  to  infection  by  micro-organisms;  according  to  Lange, 
bacteria  are  present  physiologically  in  the  lacteal  ducts  of  the  new- 
born (just  as  has  been  demonstrated  in  adult  women).  Through  trauma 
these  organisms,  which  are  harmless  as  long  as  the  epithehum  is  intact, 
wander  into  the  tissues  and  set  up  their  inflammatory  reaction.  Ulcers 
and  fissures  of  the  nipple  are  possibly  also  portals  of  entry  for  these 
bacteria. 

11—2 


18  THE   DISEASES   OF   CHILDREN 

The  diagnosis  is  easily  made.  It  is  hardly  possible  to  mistake  it 
for  physiological  lactation  and  retention  of  the  secretion,  since  with  the 
latter,  oedema,  redness  and  tenderness  are  wanting. 

The  prognosis  is  good;  complications  such  as  extensive  phlegmo- 
nous cellulitis  with  resultant  sepsis  are  very  rare  and  preventible  by 
rational  treatment.  The  gland  is  partially  destroyed  by  inflammatory 
processes  and  this  is  of  importance  for  the  female,  since  women  who 
have  gone  through  attacks  of  mastitis  as  children,  later,  in  their  puor- 
perium,  have  poorly  developed  breasts,  unsuitable  for  nursing. 

Prophylaxis  and  Treatment. — To  prevent  the  occurrence  of 
mastitis,  the  breast  of  the  newborn  infant  should  be  protected  from  all 
manner  of  trauma.  For  this  reason  expressing  the  contents  of  the 
gland  is  unquahfiedly  interdicted  and  the  secreting  gland  should  be  pro- 
tected from  pressure  by  the  apphcation  of  sterile  cotton  buffers.  With 
the  appearance  of  the  first  symptoms  of  inflammation  a  dressing  should 
be  apphed.  Gauze  compresses  soaked  in  liquor  alumini  acetatis  (P.  G.) 
diluted  8-10  times  or  in  half-strength  aqua  plumbi,  or  in  50  per  cent, 
alcohol,  are  applied  to  the  diseased  breast,  covered  with  oiled-silk  and 
fixed  by  means  of  a  binder. 

If  fluctuation  is  demonstrable,  incision  is  indicated.  The  incision 
should  be  made  as  near  the  periphery  of  the  gland  and  as  small  as  possi- 
ble, and  to  insure  the  cutting  of  the  fewest  number  of  ducts,  it  should 
run  in  a  radiating  direction  from  the  nipple.  After  incision  the  wound 
should  be  dressed  with  dermatol  or  airol,  and  sterile  gauze  or  possibly 
with  moist  dressings.  Recovery  follows  in  a  few  days.  It  is  possible 
that  the  application  of  the  suction  apparatus,  according  to  Bier,  will 
have  a  favorable  effect  in  cases  of  mastitis  of  the  newborn,  just  as  has 
been  shown  for  the  same  disease  in  mothers. 

III.    VAGINAL    HAEMORRHAGE    IN    THE    NEWBORN 

In  certain  otherwise  healthy  girls  there  occurs  on  the  fifth  or  sixth 
day  and  sometimes  later,  a  ha-morrhage,  usually  very  scant,  from  the 
vagina.  Schukowsky  found  35  such  cases  among  10,000  newborn  girls. 
Blood-stained  mucus  or  small  blood-clots  are  found  on  the  labia  of  the 
infant;  on  separating  the  labia  it  is  seen  that  the  blood  comes  from  the 
vagina  since  small  clots  and  strings  of  blood-stained  mucus  adhere  to 
the  visible  portion  of  the  vagina  as  also  to  the  hymen.  In  case  the 
haemorrhage  assumes  somewhat  greater  proportions,  blood-clots  mixed 
with  mucus  and  clumps  of  blood-stained  mucus  are  also  found  in  the 
diaper.    The  bleeding  is  never  severe  enough  to  come  in  drops. 

In  many  cases  the  bleeding  stops  in  a  day  or  two,  in  other  cases  it 
persists  for  a  week  or  recurs  after  the  pause  of  a  day.  The  bleeding 
never  disturbs  the  well-being  of  the  child;  the  fatal  cases  of  vaginal 
haemorrhage  reported  by  Doleris  must  have  been  due  to  other  causes. 


DISEASES   OF   THE    NEWBORN  19 

Inasmuch  as  they  were  attended  by  severe  constitutional  disturbances, 
they  may  have  been  due  to  sepsis. 

This  vaginal  bleeding  of  the  newborn  cannot  be  confused  with 
precocious  menstruation  which  sometimes  occurs  with  an  abnormally 
early  development  of  the  sexual  organs.  For  the  genuine  premature 
menstrual  bleeding  (menstruatio  pra'cox)  does  not  occur  in  the  first 
weeks  of  hfe,  but  later;  moreover  it  almost  always  starts  after  months 
or  years  and  at  least  recurs  for  a  few  months  more  or  less  regularly  at 
monthly  intervals;  whereas  the  condition  described  as  vaginal  haemor- 
rhage never  recurs.  Besides,  this  precocious  menstruation  is  accom- 
panied by  a  premature  development  of  all  of  the  sexual  organs,  the 
ovaries,  mamma>,  pubic  hair,  etc. 

In  rare  cases  girls  both  smaller  and  larger,  have  irregularly  recur- 
ring ha?morrhages  from  the  vagina,  which  are  due  to  malignant  neo- 
plasms. Therefore  the  vagina  must  be  carefully  inspected  in  every 
case  of  vaginal  ha-morrhage;  polypoid  sarcoma  of  the  vagina  which 
produces  no  other  symptoms  at  its  beginning,  may  thus  be  discovered. 

The  cause  of  vaginal  haemorrhage  of  the  newborn  was  inexplicable 
until  recently.  Asphyxia  and  endometritis  have  been  advanced  as  the 
etiological  factor  in  a  few  cases.  Ritter  attributed  the  genital  ha'mor- 
rhages  of  newborn  girls  to  the  same  causes  which  produce  hamorrhages 
in  other  parts  of  the  body.  Diseases  of  the  newborn,  namely,  septicae- 
mia (pyaemia)  are  so  often  accompanied  by  bleedings  that  the  vaginal 
bleeding  of  the  newborn  might  also  be  attributed  to  the  same  cause, 
the  genital  haemorrhages  being  considered  as  local  manifestations  of  an 
existing  haniophilic  diathesis.  Ritter  concluded  this  from  his  series, 
which  embraced  7  cases  of  genital  haemorrhage;  in  4  of  these,  haemor- 
rhage of  other  organs  (gastro-intestinal)  was  also  present. 

Later  investigations  show  that  Ritter's  opinion  is  not  correct.  In 
the  vast  majority  of  children  with  vaginal  Ini'morrhagc.  septicaemia  is 
not  even  to  be  considered.  Still  one  must  admit  that  according  to  Rit- 
ter's observation  vaginal  haemorrhage  may  at  times  accompany  septi- 
caemia. This  is  to  be  considered  exceptional;  for  as  a  rule  healthy 
children  are  affected. 

Zappert's  investigations  showed  that  only  hypersemia  and  diaped- 
esis  of  red  corpuscles  from  the  dilated  vessels  take  place  in  the  uterus 
and  that  signs  of  inflammation  are  wanting.  This  finding  is  in  accord 
with  Halban's  investigations  according  to  which  newborn  girls,  with 
few  exceptions,  show  changes  in  the  uterus  which  correspond  to  those 
found  in  the  premenstrual  or  menstrual  condition  of  the  uterus  in  the 
adult;  these  are:  congestion  and  subepithehal  hirmorrhages,  sometimes 
also  haemorrhages  into  the  cavity  of  the  uterus.  These  changes  are  called 
forth  in  the  sexually  mature  women  by  a  function  of  the  ovaries;  in  the 
fcEtus  and  newborn,   whose  ovaries  are  functionally  undeveloped,  the 


20  THE   DISEASES   OF   CHILDREN 

menstrual  changes  in  the  uterus  are  brought  about  by  substances,  cir- 
culating in  the  maternal  and  ftrtal  blood  during  pregnancy,  which  arise 
in  the  placenta,  probably  in  the  chorionic  epithehuni  (Halban).  Soon 
after  birth  the  mucosa  of  the  uterus  returns  to  its  normal  state  and  the 
uterus  itself  becomes  smaller;  3  weeks  post  partum  the  puerperal  invo- 
lution of  the  womb  is  completed  (Halban).  The  changes  occurring  in 
the  prostates  of  newborn  boys  (hyperaemia,  haemorrhages,  infiltrations 
around  the  glands,  secretion)  described  by  Schlachta,  are  also  classified 
under  the  reactions  of  pregnancy  which  disappear  within  the  first  2-3 
months  and  are  attributed  to  the  same  cause  as  the  manifestations  in 
the  uterus  of  newborn  girls  (Halban).  The  oedema  of  the  vulva  which 
is  seen  so  commonly  in  newborn  girls  could  also  with  probabihty  be 
attributed  to  the  active  circulating  substances  of  pregnancy. 

This  cedema  shows  itself  immediately  after  birth  or  in  the  first  days 
of  life  and  gradually  disappears.  After  this  the  labia  majora  are  wrink- 
led or  thrown  into  folds.  Similarly  an  oedema  of  the  penis  and  .scrotum 
occurs  in  newborn  boys  which  disappears  after  days  or  weeks  and  is 
entirely  meaningless.  These  cedemas  have  been  attributed  by  various 
authors,  to  stasis  during  parturition  or  to  compres.sion  of  the  veins  on 
account  of  the  intra-uterine  position  of  the  extremities.  The  fact  that 
these  cedemas  occur  also  in  children  deUvered  by  Ctesarean  section  speaks 
against  the  first-given  explanation  (Halban). 

C.   ICTERUS  NEONATORUM 

Symptoms. — Usually  before  the  end  of  the  first  or  second  day  of 
life,  less  freiiuently  not  until  the  third,  fourth  or  fifth  day,  and  only 
very  exceptionally  still  later,  the  skin  of  the  newborn  takes  on  a  yel- 
lowish tint  which  usually  appears  first  on  the  face.  The  icteric  tint 
spreads  rapidly  over  the  trunk  and  extremities.  In  many  cases,  espe- 
cially in  the  beginning,  the  physiological  hypera?mia  of  the  skin  conceals 
the  yellow  color,  which  then  only  becomes  distinct  on  pressure  of  the 
examining  finger.  Besides  the  skin,  the  sclera  as  a  rule,  but  not 
always,  is  distinctly  yellow;  here  the  icterus  sometimes  is  completely 
concealed  by  ecchymoses  or  vascular  injection.  On  "expressing"  the 
blood  from  the  mucosa  of  the  mouth,  the  yellow  color  comes  out  promi- 
nently in  the  ana>mic  area.  The  yellow  color  is  usually  specially  distinct 
in  ulcerative  processes  in  the  oral  cavity  (Bednar's  aphtha?,  etc.);  the 
secretion  of  suppurative  processes  is  also  colored  yellow. 

The  pulse  rate  in  the  newborn  is  not  influenced  by  icterus. 

The  icterus  of  older  children  is  also  usually  unaccompanied  by  a 
slowing  of  the  pulse  rate.  This  must  be  attributed  to  the  fact  that  the 
bile  of  children  contains  only  small  quantities  of  the  biliary  acids. 
Jakubowitsch  in  an  analysis  of  the  bile  of  children  found  glycocholic 
but  no  taurocholic  acid. 


DISEASES    OF    THE    XEAVHORN  21 

The  liver  and  spleen  show  no  changes.  Aftfr  the  passage  of  the 
meconium,  the  feces  take  on  the  "yolk-yellow"  or  green  color,  charac- 
teristic for  the  nursling.  The  xtrine  is  also  pale  during  the  course  of  the 
icterus.  The  examination  for  Ijile-pigment,  with  the  customary  tests, 
is  usually  negative.  Epstein,  alone,  claims  to  have  often  found  bile- 
pigment  in  icterus  neonatorum  by  using  Huppert's  test;  and,  according 
to  Cruse,  bilirubin  may  be  demonstrated  in  the  extract  after  agitating 
the  urine  with  chloroform.  Halberstam  was  also  able  to  demonstrate 
biliary  acids,  in  fact  glycocholic  acid,  in  the  urine  of  icteric  newborns. 

The  microscopic  examination  of  the  urine  during  the  perioil  of 
icterus  shows  clumps  of  a  dark  pigment;  these  were  already  knoAvn  to 
Virchow,  were  termed  "masses  jaunes"  by  Parrot  and  Robin  and  were 
identified  with  bilirubin  by  Cruse;  the  low  solvent  power  of  the  urine 
for  bilirubin  (the  urine  of  the  newborn  contains  usually  only  traces  of 
the  alkaline  phosphates  wliich  could  dissolve  the  bilirubin)  is  the  reason 
for  the  failure  of  solution  of  the  bile-pigment,  which  is  then  usually 
found  forming  clumps  with  epithelial  cells  for  a  nucleus. 

Icterus  neonatorum  is  usually  very  slight  in  degi-ee;  at  times  it  is 
quite  intense.  The  duration  of  the  jaundice  varies  from  two  daj's  to 
three  weeks;  the  intensity  of  the  discoloration  determines  this.  In  the 
majority  of  cliildren  the  icterus  disappears  or  becomes  indistinct  in 
from  six  to  eight  days.  In  case  the  icterus  increases  in  intensity  toward 
the  end  of  the  second  or  even  the  third  week,  we  may  well  assume  that 
we  are  not  dealing  with  that  variety  of  icterus  termed  icterus  neonatorum, 
but  rather  with  an  icterus  due  to  some  other  and  usually  more  serious 
disease.  Tliis  disease,  in  most  cases,  is  sepsis,  in  the  cUnical  picture  of 
wliicli  icterus  plays  a  prominent  role.  In  case  sepsis  can  be  excluded 
and  if  toward  the  end  of  the  first  month  of  life  the  icterus  becomes  more 
pronounced,  consideration  must  be  directed  toward  the  possible  pres- 
ence of  an  obstructive  jaundice.  The  latter  condition  is,  in  general,  rare 
in  the  newborn  and  may  be  due  to  various  causes,  one  of  wMch  is  a 
congenital  obliteration  of  the  hile-ducts,  with  wliich  condition  the  feces 
are  only  slightlj'^  colored  and  the  icterus  increases  in  intensity  from  day 
to  day  until  the  skin  takes  on  a  yellowish  green  color. 

In  cases  of  obstructive  or  of  septic  icterus,  one  is  usually  able  to 
demonstrate  bile-pigment  in  solution  in  the  urine;  this  ability  to  put 
bile-pigment  in  solution  e\-idently  depends  upon  changes  in  the  metabo- 
lism, which  are  brought  about  by  the  disease  and  wliich  change  the  com- 
position of  the  urine.  A  positive  finding  -wiih.  GmeUn's  test  or  with  any 
other  test  for  bile-pigment,  cannot  be  employed  for  the  purpose  of 
excluding  icterus  neonatorum,  since  in  the  latter  disease  the  reaction 
is  also,  at  times,  positive. 

Occurrence. — Icterus  neonatorum  occurs,  with  few  exceptions,  in 
all  cliildren,  with  varying  intensity.     Reports  vary  widely  concerning 


22  THE   DISEASES   OF   CHILDREN 

its  frequency;  thus  Seux  declares  that  15  per  cent.,  and  Bouchut  33?, 
per  cent,  of  all  newborn  children  have  icterus,  whereas,  according  to 
Poruk  79.9  per  cent.,  according  to  Cruse  84. 4  per  cent,  and  according  to 
Breschet  all  newliorn  cliildren  become  icteric.  My  own  observations 
are  most  nearly  in  accord  with  those  of  Cruse.  The  icterus  is  especially 
pronounced  in  premature  cliildren,  just  as,  in  general,  the  body  weight 
and  the  intensity  of  the  icterus  are  inversely  proportional  (Cruse).  The 
icterus  is  said  to  be  especially  intense  in  the  cliildren  that  show  shortly 
after  birth  a  pronounced  congestion  of  the  skin.  Cliildren  in  whom  the 
umbilical  cord  is  tied  early  are  said  to  have  icterus  less  often  than  those 
in  whom  the  cord  is  not  tied  till  the  cessation  of  its  pulsations.  The 
children  of  primiparse  are  said  to  become  more  intensely  icteric  than 
cliildren  of  multipara^  (Kehrer). 

Pathological  Anatomy. — Post-mortem  examinations  in  cases  of 
icterus  neonatorum  are  to  some  extent  rendered  less  conclusive,  because 
it  is  always  some  complication  which  has  lead  to  the  fatal  termination. 
Among  the  findings  wliicli  must  be  ascribed  to  icterus  neonatorum,  the 
observation  that  the  liver  is  often  not  especially  yellow  or  else  only  in 
spots,  deserves  first  mention.  This,  however,  applies  also  to  other  forms 
of  icterus.  The  finding  of  biUrubin  crystals  in  the  kidneys  (Meckel, 
Virchow's  hsematoidin-infarct)  is  peculiar;  these  are  found  in  the  form 
of  bunches  of  needles  or  rhombic  platelets,  in  the  tips  of  the  papillae. 
Orth  found  these  crystals  in  every  case  of  icterus  neonatorum  and  more- 
over not  only  in  the  kidneys  but  also  in  the  blood,  in  the  adipose  tissues, 
the  brain  and  other  organs.  In  examining  the  cadavers  of  still-born 
infants,  or  those  of  children  dj'ing  shortly  after  birth,  Neumann  found 
bilirubin  crystals  in  the  fat  cells  of  the  peritoneum  and  in  the  afferent 
blood  vessels.  The  precipitation  of  bilirubin  in  the  fatty  tissue  may  be 
due  to  the  withdrawal,  by  the  fatty  acids  in  the  fat  cells,  of  the  alkali, 
wliich  is  the  solvent  for  bilirubin. 

For  the  rest,  one  finds,  at  autopsy,  a  yellow  discoloration  of  most  of 
the  internal  organs;  this  is  especially  marked  in  the  serous  membranes, 
the  intima  of  the  vessels,  in  exudates  and  transudates  and  is  not  notice- 
able in  the  spleen  or  kidneys. 

Pathogenesis. — The  causes  of  icterus  neonatorum  have  not  yet 
been  clearly  established.  It  can  be  said  that  the  views  concerning  the 
pathogenesis  of  icterus  neonatorum  are,  in  general,  parallel  to  the 
theories  of  icterus  in  the  adult  and  to  those  of  experimental  icterus. 

If  we  accept  the  dictum  of  Stadelmann  "without  a  hver  no 
icterus,"  then  all  theories  which  seek  to  explain  icterus  without  in- 
volvement of  the  liver  must  be  discarded.  We  know  from  the  celebrated 
experiments  of  Naunyn  and  Minkowski  that  the  liver  is  not  only  the 
organ  for  the  excretion  of  the  bile  but  also  for  its  elaboration.  In  the 
liver  arise  the  characteristic  components  of  the  bile,  the  bile-pigment  as 


DISEASES   OF   THE    XEWBORX  23 

well  as  the  biliary  acids.  It  is  true  that  occasionally  bilirubin  arises, 
without  involvement  of  the  liver,  from  the  blood-pigment,  (e.g.,  in  haem- 
orrhages into  the  tissues)  but  icterus  has  never  been  observed  in  this 
connection.  The  forms  of  icterus  in  which  biUary  acids  are  demonstrated 
in  the  urine  must  unqualifiedly  be  attributed  to  the  resorption  of  bile 
in  the  liver.  This  presence  of  the  biliary  acids  has  been  demonstrated 
for  icterus  neonatorum  and  moreover  not  only  in  the  urine  (Halberstam) 
but  also,  earlier,  in  the  pericardial  fluid  (Birch-Hirschfeld  and  Hof- 
meister). 

Therefore,  all  theories  wliich  have  sought  to  explain  icterus,  by 
means  of  a  hyperaemia  of  the  skin,  capillary  haemorrhage  and  the  like, 
have  only  a  historical  interest.  Just  as  little  do  those  theories  deserve 
consideration,  wliich  would  attribute  the  icterus  to  haemolysis  and  trans- 
formation of  blood-pigment  to  bile-pigment  within  the  circulatory 
apparatus  (hematogenous  jaundice,  Neumann,  Violet,  and  others).  The 
finding  of  bihary  acid  in  the  urine  absolutely  excludes  such  a  theory. 
However,  one  theory,  that  of  Luincke,  wliich  explains  icterus  without 
liver,  is  not  demolished  by  this  fact;  according  to  Luincke,  the  biliary 
components  are  resorbed  from  the  meconiuiu;  since,  however,  the  bil- 
iary acids  and  pigments  are  normally  excreted  in  the  liver  during  their 
passage  through  the  portal  circulation  and  therefore  do  not  enter  the 
general  circulation,  Luincke  assumes  that  a  portion  of  the  blood  in  the 
mesenteric  veins,  laden  with  the  biliar}'  constituents  of  the  meconium, 
passing  through  the  as  yet  unclosed  ductus  Arantii,  enters  the  vena 
cava  ascendens  and  thus  into  the  general  circulation  without  passing 
through  the  liver.  Against  this  theory  it  must  be  noted  that,  according 
to  Meckel,  the  ductus  Arantii  in  the  newborn  is  usually  hardly  passable 
by  a  sound,  further  that  meconium  is  acid  in  reaction,  whereas  bilirubin 
is  soluble  in  alkahne  fluids  and  finally  that  the  expulsion  of  the  meconium 
during  parturition,  for  example  with  asphyxia,  does  not  hinder  the 
occurrence  of  icterus.  We  must,  therefore,  look  to  the  liver  as  the  place 
of  origin  for  icterus.  The  most  satisfactory  theory  would  be  one  that 
would  connect  icterus  neonatorum  with  a  demonstrable  stasis  of  bile. 
To  this  end,  Peter  Franck  assumed  a  closure  of  the  ductus  choledochus 
by  means  of  the  meconium;  Virchow,  by  means  of  a  plug  of  mucus; 
and  Cruse-,  through  cast  off  epithelium;  but  these  assumptions  do  not 
accord  -nith  the  facts;  no  more  is  the  theory  of  Birch-Hirschfeld  right, 
according  to  which  an  a^lema  of  the  capsule  of  Ghsson  is  the  cause  of 
the  stasis;  the  a>dema  which  Birch-Hirschfeld  found  was  not  verified 
by  other  investigators  and  must  be  attributed  to  complications  (pneu- 
monia, etc.).  By  anatomical  examinations  of  the  Uver,  Bouchut's 
hypothesis  of  a  hepatitis  and  Epstein's  theory  of  a  catarrh  of  the  finer 
gall-ducts  have  been  demohshed.  The  assumption  of  Ritter  and  Ep- 
stein that  icterus  of  the  newborn  is,  in  the  majority  of  instances,  of  a 


24  THE    DISEASES   OF   CHILDREN 

septic  nature,  seems,  in  the  light  of  cHnical  observation,  untenable; 
the  well-being  of  icteric  children  and  the  absence  of  any  other  symptoms 
of  sepsis  speak  against  any  such  assumption. 

Endeavor  has  been  made  to  attribute  icterus  neonatorum,  in 
accord  with  the  extensive  experiments  of  Stadelmann,  Affanassiew, 
Tarchanoff  and  others,  to  a  polycholia  or  more  correctly  a  pleo- 
chromia,  which  arises  as  the  result  of  the  destruction  of  countless 
erythrocytes  during  the  first  days  after  birth  (Hofmeier,  Sill)ermann, 
and  others).  However,  the  supposition  that  during  the  first  days 
of  life  there  is  an  extensive  disintegration  of  red  cells  has  been 
refuted  and  with  this  refutation  the  hypothesis  of  a  plcochroic 
icterus  becomes  untenable. 

Investigations  have  shown  that  the  blood  of  the  newborn  is,  at 
birth,  richer  in  corpuscles  and  hipmoglolnn  than  that  of  the  adult.  A 
count  of  si.x  to  seven  million  erythrocytes  in  a  cubic  millimetre  is  not 
uncommon.  This  count  sometimes  increases,  during  the  first  two  or 
three  days  of  life  and  then  falls  (Lepine,  Hayem,  and  others).  The  ex- 
planation of  this  has  already  been  given  by  these  writers.  The  increase 
and  decrease  in  the  number  of  cells  is  only  apparent  and  is  brought 
about  by  variations  in  the  quantity  of  the  blood  plasma.  Cohnstein  and 
Zuntz  furnished  the  experimental  proof. 

In  nursling  animals  showing  variations  in  the  number  of  cells  per 
cubic  millimetre,  similar  to  those  of  newborn  children  during  the  first 
days  of  life,  determinations  of  the  total  number  of  red  cells  have  indi- 
cated that  their  number  increases  from  the  first  day  of  life  but  never 
decreases.  Microscopic  examination  and  the  determinations  of  the  re- 
sistence  of  the  red  cells  have  shown,  contrary  to  Silbermann,  the  absence 
of  the  products  of  red  cell  destruction  (Fischl,  Knopfelmacher). 

The  destruction  of  red  cells  in  the  newborn  has  also  been  connected 
with  the  "physiological  transfusion"  which  every  newborn  experiences 
at  birth.  At  birth  a  large  part  of  the  blood  present  in  the  placenta  is 
expressed  into  the  foetus;  and  when  the  cord  is  tied  off  late,  this  amount 
is  further  increased  (according  to  Budin  by  about  90  Gm.  or  3  oz).  This 
superfluous  blood  is  said  to  be  destroyed  during  the  first  days.  Hof- 
meier endeavored  to  verify  this  hypothesis  by  metabolism  experiments. 
As  far  as  the  erythrocytes  are  concerned,  it  has  been  established  that 
they  are  not  destroyed  in  abnormal  numbers. 

Two  other  peculiar  theories  rest  on  the  assumption  of  a  destruc- 
tion of  blood  corpuscles  in  the  newborn.  Silbermann  advanced  the  one: 
the  destruction  of  the  erythrocytes  is  supposed  to  lead  to  "fermentie- 
mia,"  through  this  to  capillary  stasis  and  thromboses  in  the  liver,  and 
thus  to  icterus.  Such  thromboses,  however,  have  never  been  found,  not 
even  by  Silbermann  himself.     With  this,  Silbermann's  theory  also  falls. 

Recently  Leuret  has  affirmed  anew  the  destruction  of  red  cells  in 


DISEASES   OF   TIIK   NEWBORN  25 

the  newborn.  It  is  sui)])()se(l  to  occur  in  consequence  of  the  cooling  of 
the  skin  after  birth.  Through  this,  as  in  paro.xysnial  lui'iiioglobinuria, 
there  arises  a  htrmoglobintcniia.  Leuret  found  this  but  no  one  else  has. 
The  hiTmoglobin  in  the  circuhition  is  changed,  in  the  tissues,  to  a  yellow 
coloring  matter.  Since,  however,  a  ha-nioglobinremia  does  not  exist  in 
the  newborn,  the  hypothesis  of  Leuret  must  also  be  rejected. 

Endeavor  has  also  been  made  to  connect  the  icterus  with  a  post 
partum  fall  in  the  blood  pressure  (Frerichs).  This  a.ssuiuption,  however 
falls,  since  icterus  is  especially  intense  in  asphyctic  children  and  those 
with  pulmonary  atelectases;  and  in  these  affections  the  blood  pressure 
is  increased. 

Not  more  fortunate  is  the  assumption  of  a  compression  of  the  gall- 
ducts  by  the  dilated  liver  veins,  distended  in  consecjuencc  of  the  afore- 
mentioned transfusion.  Histologic  examination  of  the  liver  in  the  new- 
born shows  the  gall-capillaries  not  compressed  but  on  the  contrary  much 
dilated  (Abramow);  my  own  observations  have  also  taught  me  this 
fact.  In  preparations  in  which  the  bile-capillaries  are  demonstrated 
according  to  the  method  of  Eppinger,  one  sees  them  mostly  varicose  and 
tortuous,  as  an  expression  of  the  extreme  fulness  of  the  gall-duct  sys- 
tem; and  moreover  this  is  present  immediately  after  birth  and  in  those 
still-born. 

In  reconsidering  the  remarks  concerning  these  various  theories, 
we  are  forced  to  the  conclusion  that  up  to  the  present  time  no  single 
theory  is  fully  compatible  with  the  facts.  Icterus  neonatorum  in  tliis 
respect  resembles  the  icterus  occurring  with  infectious  diseases  and 
with  sepsis  in  which,  similarly,  the  anatomical  examination  of  the  liver 
gives  no  explanation  of  the  resorption  of  bile. 

In  order  to  make  the  passage  of  the  bile  over  into  the  blood  com- 
prehensible, in  such  cases,  one  can  assume  with  Minkowski,  Lieber- 
meister  and  others,  that  we  are  dealing  with  a  functional  secretory 
anomaly  of  the  liver  cells;  according  to  Jlinkowslci,  in  such  cases  the 
liver  cell  has  lost  the  faculty  of  sending  the  bile-pigment  and  l)iliary 
acids  into  the  gall-ducts  alone  and  gives  these  substances  up  to  the 
blood-capillaries  also.  According  to  Pick,  the  bile  is  directed  into  the 
lymphatic  system.  According  to  Abramow,  an  increased  secretion  of 
bile  gives  rise  to  this  secTi-etory  disturbance;  the  liver  cell  produces  bile 
profusely  but  its  excretory  energy  is  not  able  to  overcome  the  abnor- 
mally high  pressure  in  the  bile-capillaries  and  consequently  the  bile  is 
turned  into  the  blood-capillaries  by  the  cell.  The  overloading  of  the 
blood-capillaries,  the  passive  congestion,  are  the  causes  for  the  assumed 
functional  disturbance  of  the  liver  cell,  wliich  Abramow  terms  "asthenic 
polychoUa." 

It  is  my  belief  that  the  causes  of  icterus  neonatorum  lie  in  the  over- 
filling of  the  bile-capillaries  in  the  foetus,  with  rather  tenacious  bile,  and 


26  THE   DISEASES   OF   CHILDREN 

in  the  lively  production  of  bile  by  the  liver  cell  immediately  after  birth, 
in  consequence  of  the  rich  supply  of  blood.  The  newly-formed  bile 
cannot  flow  out  through  the  over-filled  bile  capillaries  and,  therefore, 
passes  from  the  liver  cell  into  the  blood-capillaries.  Accordingly,  icterus 
neonatorum  is  a  physiological  manifefitation.  It  offers  the  most  favor- 
able prognosis  and  leads  to  no  complications.  The  statement  that  it 
exerts  an  effect  on  the  general  health  of  the  child,  has  not  yet  been  sub- 
stantiated. It  has  been  stated  (Schaeffcr)  that  intensely  icteric  chil- 
dren especially  lose  weight  during  the  first  days  of  life.  It  is  possible  that 
a  considerable  resorption  of  bile  and  biliary  acids  can  be  held  responsi- 
ble for  this. 

Medical  intervention  in  cases  of  icterus  neonatorum  is  in  no  sense 
indicated. 

III.     DISEASES  OF  THE  NAVEL 

A.     ANATOMICAL  AND  PHYSIOLOGICAL  INTRODUCTION 

At  birth  the  umbilical  cord  forms  the  connecting  link  between  the 
body  of  the  embryo  and  the  placenta.  It  is  a  cord,  about  as  tliick  as 
the  little  finger,  twisted  in  many  spirals  and  consists  fundamentally  of 
the  jelly  of  Wharton,  an  embryonic  mucous  tissue  which  contains 
countless  connective  tissue  and  elastic  fibres  embedded  in  its  colloid 
matrix.  The  cord  is  covered  with  amnion  throughout  its  length;  this 
terminates  abruptly  about  1  cm.  above  the  fa^al  abdominal  wall, 
leaving  the  proximal  portion  covered  with  skin.  The  umbilical  cord 
contains  no  capillaries;  only  at  its  point  of  entrance  into  the  navel  does 
there  appear,  in  the  peritoneal  tissue,  a  rich  capillary  network,  whose 
branches  extend  on  to  the  intra-abdominal  portion  of  the  umbilical 
vessels  and  send  numerous  twigs  upward  into  the  collar  of  skin  cover- 
ing the  lowest  portion  of  the  cord;  here,  at  the  boundary  between 
amnion  and  skin,  they  form  a  vascular  circle  (Hyrtl). 

At  birth  the  umbilical  cord  contains: 

1.  The  two  umbilical  arteries  which  conduct  the  blood  from  the 
foetal  body  to  the  placenta.  They  extend  to  the  navel  on  either  side 
from  the  common  iliac  artery;  they  are  thick  walled  vessels  and  like  the 
umbilical  cord,  are  wound  in  spirals. 

2.  The  umbihcal  vein  which  conducts  the  blood  from  the  placenta 
to  the  inferior  vena  cava.  It  passes  through  the  cord  to  the  body  of 
the  child,  thence  through  the  navel  ring  along  the  abdominal  wall  to 
the  left  branch  of  the  portal  vein  which  conducts  the  placental  blood 
through  the  ductus  venosus  Arantii  into  the  inferior  vena  cava  of  the 
foetus. 

3.  Strands  and  epithelial  remains  representing  a  portion  of  the 
ductus  om phalomesentericvs  (the  vitelline  duct),  which  disappears  at  the 
first  month  of  foetal  life. 


DISEASES   OF   THE    NEWBORN 


27 


4.  Clumps  of  cells  arising  from  the  involuted  allantoic  duct.  The 
extra-abdominal  portion  of  the  allantois  undergoes  retrograde  meta- 
morphosis in  the  first  weeks  of  fo-tal  life;  leaving  only  a  few  epithelial 


Fig.  4. 


Placenta 

Diagrammatic  view  of  the  foetal  circulatory  system. 

clumps,  whereas  the  abdominal  portion  remains  as  the  urachus;  it  is 
normally  a  solid  strand  or  often  partially  or  totally  patent  and  Uned 
with  epitheUum  and  after  birth  becomes  the  median  umbihcal  Ugament. 
Immediately  after  birth,  usually  after  the  cessation  of  pulsations 
in  the  umbilical  arteries,  the  cord  is  cut;  among  ci\alized  peoples,  after 


28  THE  DISEASES   OF   CHILDREN 

previous  ligature.  There  remains  adhering  to  the  body  of  the  child  a 
portion  of  the  cord,  one  or  more  centimetres  in  length,  which  under- 
goes mummification  during  the  first  days  of  life.  On  the  fourth  to  ninth 
day,  rarely  earlier  or  later,  it  separates  from  its  attachment  with  a  slight 
inflammatory  reaction.  The  separation  occurs  later  with  thick  ccdem- 
atous  cords  and  in  premature  children  than  with  thin  cords  and  strong 
children.  The  skin  of  the  abdominal  wall  which  covers  the  beginning 
of  the  cord  for  a  distance  of  l-lj  cm.,  rolls  in  at  the  same  time  and 
thus  forms  the  umbilical  fold,  the  upper  half  of  which  is  smaller  than 
the  lower.  The  navel  fold  usually  hides  the  base  of  the  navel  so  com- 
pletely that  one  is  unable  to  see  its  epidermization  without  separating 
the  folds.  The  healing  of  the  navel  wound  progresses,  with  scant  secre- 
tion, by  epidermization  from  the  periphery  toward  the  centre. 

The  healing  of  the  navel  wound  and  its  pathological  disturbances 
depend  closely  on  the  pccuhar  construction  of  the  umbihcal  vessels. 
The  umbilical  arteries  differ  materially  in  structure  from  the  other  ves- 
sels of  the  body.  They  possess  a  single  layer  of  endothelium  and  a  highly 
developed  musculature.  The  latter  is  arranged  in  two  layers.  The  inner 
layer  is  composed  of  longitudinally  disposed  fibres  richly  mingled  with 
elastic  fibres  and  connective  tissue.  The  outer  layer  is  still  thicker,  con- 
tains principally  circular  fibres  and  is  poor  in  elastic  substance  and 
connective  tissue;  occasionally  besides  the  circular  fibres  it  contains 
some  longitudinal  fibres.  Further,  the  umbilical  arteries  are  enveloped 
in  a  dense  mantle  of  embryonic  connective  tissue;  this  forms  the  ad- 
ventitia  of  the  arteries  and  accompanies  them  downwards  through  the 
navel  ring  into  the  abdominal  cavity  as  far  as  the  summit  of  the  bladder. 

Examination  of  the  lumen  of  the  umbilical  artery  in  the  constricted 
state  shows  it  to  be  very  narrow  and  in  the  form  of  the  letter  Y;  this 
comes  from  the  inward  bulging  of  the  vessel  walls  as  a  result  of  the 
contraction  of  its  longitudinal  fibres;  this  contraction  almost  com- 
pletely closes  the  lumen  of  the  vessel,  an  open  lumen  being  found  for 
only  short  distances.  This,  however,  only  holds  good  for  the  umbilical 
arteries  of  children  born  viable,  whereas  these  bulgings  never  occm-  in 
the  arteries  of  still-born  infants.  The  umbilical  vein  forms  a  smooth 
wide  tube  and  possesses  likewise  a  single  layer  of  endothelium;  its  mus- 
culature runs  in  various  directions  but  is  for  the  most  part  circularly 
disposed.  There  is  a  band  of  elastic  fibres  beneath  the  endothelium 
(Bondi);  the  muscular  coat  is  traversed  by  wavy  connective  tissue  and 
by  a  few  elastic  fibres.  In  its  extra-abdominal  portion  the  umbilical 
vein  also  possesses  a  well  developed  adventitia  of  embryonic  connective 
tissue,  which  is  wanting  in  its  abdominal  portion,  where  the  vessel  lies 
rather  free  in  its  surroundings   (Herzog). 

At  the  moment  of  birth  as  a  consequence  of  the  opening  of  the  pul- 
monary circulation  the  blood  pressure  in  the  aorta  and  in  the  entire 


DISEASES   OF   THE    NEWBORN  29 

greater  circulation,  including  the  umbilical  arteries,  falls;  for  now,  in- 
stead of  both  sides  of  the  heart,  only  the  left  side  is  active  in  pumping 
the  blood  through  the  systemic  circulation.  The  umbilical  arteries  grad- 
ually contract  throughout  their  extent;  tlie  contraction  commences  in 
the  peripheral  portion  and  extends  to  tlie  abdominal  portion  and  is 
materially  assisted  by  the  mechanical  irritation  arising  from  the  cutting 
of  the  cord  and  perhaps  also  by  its  cooling;  thus  the  arteries  are  so  well 
contracted  that  in  the  vast  majority  of  cases  no  bleeding  takes  place 
after  the  cutting  of  the  cord  even  should  no  ligature  be  applied.  The 
vein  also  contracts;  its  contained  blood  flows  out,  and  under  normal 
conditions  shortly  after  birth  it  is  either  empty  or  contains  only  a  thin 
clot  in  the  neighborhood  of  the  navel.  Since  the  cord,  as  stated,  possesses 
no  blood  vessels  of  its  own  for  its  nourishment  its  death  begins  with  the 
cessation  of  the  placental  circulation.  Tliis  occurs  normally  tlirough  a 
process  of  mummification,  which  is  assisted  bj'  all  factors  promoting 
evaporation,  especially  by  warmth  and  dry  air. 

The  dessication  begins  at  various  points  on  the  cord  and  is  as  a 
rule  completed  by  the  third  day.  Then  the  cord  is  converted  into  a  dark, 
ropy,  flat  body;  the  drying  out  process  takes  place  throughout  the 
cord  except  at  its  base  and  here  the  cord  stays  moist  over  a  few  milli- 
metres of  its  extent  and  disintegrates  gradually.  Simultaneously  changes 
occur  in  the  navel,  corresponding  to  a  demarcating  inflammatory 
process;  these  cause  the  separation  of  the  cord.  The  cylinder  of  skin 
which  covers  the  lower  part  of  the  cord  swells  and  becomes  red;  its 
border  frees  itself  slightly  from  the  cord  and  begins  to  roll  in  upon  itself; 
in  this  way  there  is  formed  between  the  cylinder  of  skin  and  the  dessi- 
cated  cord,  a  furrow  wliich  is  filled  with  greasy  material  composed  of 
tissue  detritus,  pus  cells,  and  bacteria.  On  liistological  examination  the 
capillaries  of  the  network  under  the  skin  covering  the  cord  are  widened 
and  collections  of  leucocytes  are  found  in  the  skin  and  in  the  jelly  of 
Wharton.  The  cord  gradually  loosens  at  its  base,  remaining  longest 
attached  at  the  blood  vessels.  Occasionally  the  stumps  of  the  vessels 
protrude  several  millimetres  out  of  the  base  of  the  wound  after  the  cord 
has  separated.  Meanwhile  the  infolding  of  the  skin-cylinder  progresses 
gradually  and  after  the  separation  of  the  cord,  the  small  sldn  wound, 
usually  covered  with  a  secretion  or  crust,  lies  in  a  little  funnel.  At  the 
same  time  epidermization  starts  at  the  periphery  and  becomes  com- 
plete about  three  weeks  after  birth. 

Shortly  after  birth  the  process  of  involution  begins  in  the  abdom- 
inal portion  of  the  umbilical  vessels.  Tlie  peripheral  portions,  at  the 
navel  itself,  become  involved  in  the  infiammatory  processes  taking 
place  at  the  navel;  their  walls  are  invaded  by  round  cells  and  their 
lumina  closed  by  small-celled  infiltration.  The  thrombi,  which  are  reg- 
ularly found  in  the  arteries  seldom  form  in  the  veins.    The  intima  prolif- 


30  THE   DISEASES   OF  CHILDREN 

erates  and  gradually  becomes  converted  into  connective  tissue  (arteritis 
obliterans).  In  the  third  week  of  life  degeneration  of  the  media  occurs; 
the  muscularis  seems  shredded  and  is  permeated  with  capillaries,  its 
structure  becomes  indistinct  and  the  nuclei  stain  feebly;  later  the  mus- 
cular coat  disappears,  being  replaced  by  connective  tissue.  The  veins 
and  arteries  usually  remain  passable  for  a  tliin  sound  for  months  and 
occasionally  for  years  after  birth. 

The  adventitia  of  the  umbilical  arteries  furnishes  the  material  for 
the  formation  of  a  dense  connective  tissue  and  tliis  attaches  the  arteries, 
as  well  as  the  connective  tissue  strands  resulting  from  their  involution, 
to  the  navel  ring  and  to  its  lower  half.  Thus  the  lower  half  of  the  ring  is 
closed  by  a  strong  buttress  of  connective  tissue,  the  fibres  of  which  ex- 
tend into  the  skin  of  the  navel.  Conditions  are  however  different  in  the 
upper  half  of  the  navel  ring.  Only  a  few  loose  strands  of  connective 
ti.ssue  form  around  the  umbilical  vein.  The  vein,  after  its  fibrous  meta- 
morphosis, draws  over  to  the  connective  tissue  bolster  which  is  formed 
from  the  adventitia  of  the  arteries  and  thus  there  ensues  a  defect  in  the 
upper  half  of  the  navel,  between  the  upper  half  of  the  ring  and  the  con- 
nective tissue  strands  of  the  vein  which  are  drawn  over  to  the  arteries; 
this  defect  is  covered  underneath,  toward  the  abdomen,  by  only  the 
thinnest  of  connective  tissue  layers  and  thus  offers  a  place  of  lessened 
resistance  against  the  impact  of  the  abdominal  contents  on  crjdng  or 
straining.     Herzog  designates  this  defect  the  "canalis  umbilicalis." 

Some  writers  teach  that  the  media  and  intima  of  the  umbilical 
vessels  retract,  so  that  in  the  upper  portion,  hing  nearest  the  navel, 
only  the  adventitia  changed  into  connective  tissue,  remains  attached. 
This  retraction,  however,  is  disputed  by  well-known  authorities  (Herzog, 
Kockel). 

After  its  involution  the  navel  appears  as  a  defect  in  the  abdominal 
wall,  closed  by  connective  tissue  tightly  in  its  lower  half,  insufficiently 
in  its  upper  portion.  The  fascia  umbilicalis  (the  portion  of  the  transver- 
salis  fascia  lying  underneath  the  navel)  extends  beneath  the  connective 
tissue  and  below  this  is  peritoneum.  The  umbilical  vessels  are  changed 
to  tense  fibrous  baiids.  From  the  vein,  the  ligamentum  teres  is  formed, 
passing  along  the  anterior  abdominal  wall  in  the  free  margin  of  the  fal- 
ciform ligament  of  the  liver  until  it  reaches  the  median  incisure.  That 
part,  alone,  of  the  umbilical  vein  whicli  connects  with  the  left  branch 
of  the  portal  vein  normally  remains  patent,  and  persists  as  a  branch  of 
the  portal  vein,  being  traversed  by  the  blood  in  a  opposite  direction  to 
that  in  fcetal  life. 

The  umbilical  arteries,  after  their  obliteration  and  conversion  into 
connective  tissue,  form  the  lateral  umbilical  ligaments,  which  extend 
from  the  lateral  pehic  wall  to  the  anterior  abdominal  wall  and  running 
properitoneally  pass  to  the  umbilicus. 


DISEASES   OF  THE   NEWBORN  31 

B.  CONGENITAL  ANOMALIES  OF  THE  UMBILICAL  REGION 
(See  Plate  6) 

1.  Amniotic  Navel. — Normally  the  skin  of  the  abdominal  wall  ex- 
tends for  about  1  cm.  over  the  umbilical  cord,  the  base  of  which  it  cylin- 
drically  encircles.  In  very  rare  cases  the  skin  is  lacking  over  the  lower 
part  of  the  cord  and  the  adjacent  abdominal  wall,  so  that  the  amnion  not 
only  extends  over  the  lowest  part  of  the  cord  but  also  spreads  out  over 
the  skin  defect  as  a  delicate,  transparent  membrane. 

The  navel  ring,  the  fibrous  tissue,  abdominal  muscles  and  peri- 
toneum are,  however,  normally  developed.  The  disc  of  amnion  becomes 
dessicated  like  the  cord,  turns  dark  in  color  and  separates  after  a  few 
days.  The  skin-defect  heals  by  granulation  and  scar-formation.  The 
life  and  progi-ess  of  the  cliild  are  not  influenced  by  tliis  anomaly. 

2.  Cutis  Navel. — (Skin  navel,  cutaneous  umbilicus). — A  not  un- 
common anomaly  occurs  when  the  abdominal  skin  is  not  drawn  in  like 
a  funnel,  after  the  separation  of  the  cord,  and  thus  the  formation  of  the 
navel-folds  is  wanting.  Then  there  appears  in  the  umbilical  region  a 
projecting  cylinder  of  skin,  about  l-lj  cm.  in  length,  at  the  apex  of 
wliich  the  umbilical  wound  is  found.  The  wound  heals  and  the  skin 
cylinder  persists.  This  anomaly  has  been  explained  by  assuming  that 
the  abdominal  skin  extends  for  an  abnormally  great  distance  over 
the  cord  (Widerhofer).  However,  it  is  more  probable  that  in  a  large 
number  of  instances,  the  cutis  navel  does  not  arise  in  tliis  waj',  but 
rather  because  of  the  failure  of  the  physiologic  infolding  of  the  free 
margin  of  the  skin  of  the  cord,  which  should  follow  the  separation 
of  the  cord.  Umbilical  hernia  occurs  frequently  in  such  children. 
With  the  gi'owth  of  the  child  the  superfluous  skin  of  the  umbilicus 
is  drawn  upon  for  the  covering  of  the  abdomen  so  that  it  gradually 
disappears  completely. 

3.  Hernia  of  the  Umbilical  Cord. — (Congenital  umbilical  hernia; 
hernia  funiculi  umbilicalis). —  In  the  sixth  to  tenth  week  of  foetal  life 
the  umbilical  cord  contains  one  or  more  loops  of  intestine,  wliich  later 
in  foetal  life  are  drawn  back  into  the  abdominal  ca^^tJ^  The  persis- 
tence of  intestinal  coils  and  other  abdominal  organs  outside  of  the  body 
cavity  constitutes  a  failure  of  development  wliich  should  here  be  con- 
sidered on  account  of  its  frequency  and  the  good  results  acliieved  by- 
proper  surgical  treatment. 

Symptoms  and  Course. — The  umbilical  region  in  the  newborn  is 
found  occupied  by  a  swelling  which  is  half-globular,  egg-shaped,  or 
pear-shaped.  Its  size  varies;  tumors  from  the  .size  of  a  nut  to  that  of 
a  child's  head  have  been  found.  They  have  a  liluish-white  transparent 
covering  which  is  continuous  with  that  of  the  cord;  the  cord  is  not 
usually  attached  to  the  middle  of  the  tumor  but  rather  to  its  lower  half. 
The  covering  of  the  mass  is  sharply  defined  from  the  abdominal  skin 


32 


THE   DISEASES   OF   CHILDREN 


which  surrounds  its  base.  If  the  covering  is  transparent  one  is  able  to 
recognize  the  intestinal  coils  or  the  other  abdominal  organs.  The  cov- 
ering is,  however,  usually  thickened  in  spots  and  sometimes  in  its  en- 
tirety. The  delicate  transparent  covering  is  formed  by  the  amnion  and 
the  parietal  peritoneum.  The  thickened  areas  result  from  the  spread- 
ing of  Wharton's  jelly  over  the  covering  of  the  tumor  and  sometimes 
from  fibrous  thickening  following  an  inflammatory  process.  On  palpa- 
tion, the  tumor  is  of  a  soft  consistency  containing  harder  masses  within. 
It  is  sometimes  attached  to  the  abdomen  by  a  broad  base  and  some- 
times it  has  a  pedicle.  If  the  child  lives  a  few  hours  or  days,  the  cover- 
ing becomes  opaque  and  inflammatory  signs  ensue  at  the  border  of  the 
abdominal  skin;  soon  the  covering  of  the  mass  dries  up  at  the  same 
time  as  the  cord  and  then  it  separates.    In  very  rare  cases  (and  especially 


Hernia  of  the  umbilical  cord  containing  the  liver  and  intestines. 

where  the  rupture  is  small)  the  hernial  contents  may  return  into  the 
abdominal  cavity;  the  abdominal  cavity  is  then  closed  by  granulations 
and  thus  the  hernia  is  cured;  or  else  the  exposed  parts,  after  separation 
of  the  tumor  covering,  become,  as  a  consequence  of  an  active  inflamma- 
tion, covered  with  granulations  which  gradually  undergo  epidcrmiza- 
tion  and  the  healing  of  the  hernia  is  thus  brought  about.  These  are, 
however,  very  rare  occurrences.  In  the  vast  majority  of  instances  peri- 
tonitis occurs  after  the  separation  of  the  hernial  sac  or  before,  and  death 
follows. 

With  very  large  hernia?,  or  in  difficult  deliveries,  the  sac  sometimes 
ruptures  during  labor  and  the  child  is  born  with  extruded  viscera.  In 
some  cases  gangrene  of  the  intestines  and  the  formation  of  a  fa?cal  fistula 
occur  even  within  the  first  days;  in  such  cases  the  accompanying  peri- 
tonitis results  fatally. 

Pathogenesis. — The  occurrence  of  hernia  of  the  cord  is  generally 


DISEASES    OF    THE    NEWBORN  33 

attributed  (Oken,  Ahlfeldt)  to  the  failure  of  tlie  intestinal  coils,  nor- 
mally present  in  the  cord  in  the  second  and  third  fcrtal  months,  to  re- 
turn to  the  abdominal  cavity  before  its  complete  closure.  The  cause 
of  this  lies,  according  to  Ahlfeldt,  in  the  behavior  of  the  ductus  omphalo- 
entericus,  the  vitelline  duct,  which  in  the  second  fcetal  month  extends 
from  the  yolk-sac  through  the  cord  to  the  intestinal  tube.  Normally 
this  duct  becomes  thinner  and  tliinnor  and  finally  atrophies  completely. 
The  intestinal  coils  leave  the  cord  and  fall  Imck  into  the  abdominal  cav- 
ity before  the  abdominal  wall  closes  down  to  the  opening  necessary  for 
the  passage  of  the  structures  of  the  cord.  However,  the  return  is  hin- 
dered in  those  cases  where  the  ductus  omphalomesentericus  either  does 
not  disappear  at  all  or  else  where  it  does  not  disappear  until  the  cavity 
is  closed  down  to  the  umbilical  ring,  the  opening  for  the  vessels  of  the 
cord  and  the  urachus.  It  is  difficult  to  explain  the  origin  of  those  her- 
nia' in  wliich  the  liver,  with  or  without  the  intestine,  forms  the  hernial 
contents. 

Ahlfeldt's  explanation  might  suffice,  in  the  cases  where  the  liver 
and  other  organs,  together  with  intestines  he  in  the  amniotic  covering; 
on  account  of  the  extrusion  of  the  intestinal  coils  there  is  more  room  in 
the  abdomen  for  the  other  organs;  these  are,  therefore,  less  tightly  held 
in  place,  become  more  movable  and  fall  into  the  hernial  sac.  Possibly 
the  prolapse  of  the  liver  has  some  connection  with  the  excessive  growth 
of  this  organ  and  especially  of  its  left  lobe  (Tandler). 

According  to  Aschoff  one  can  not  explain  these  hernise,  nor  hernia' 
of  the  liver  alone,  nor  any  umbilical  cord  hernias,  by  a  persistence  of 
the  ductus  omphalomesentericus;  but  rather  in  a  totally  different  way. 
Aschoff  assumes  that  in  these  cases  the  liver  is  not  developed  laterally 
in  the  abdomen  under  the  closed  abdominal  wall,  as  in  the  normal 
development;  but  rather  in  an  abnormal  location.  The  abnormal  posi- 
tion of  the  umbilical  veins  in  these  cases  argues  for  tliis  theory.  This 
explanation  may  be  considered  as  satisfactory  for  the  rare  cases  in  which 
the  liver  alone  is  found  in  the  hernial  sac. 

The  prognosis  of  hernia  funiculi  umbilicalis  was  formerly  an  abso- 
lutely unfavoral)le  one.  However,  since  the  institution  of  the  operative 
treatment,  the  prospect  of  the  survival  of  the  child  is  very  promising, 
if  the  operation  is  performed  as  early  as  possible.  According  to  the 
statistics  of  Kindt,  out  of  65  cases  treated  by  operation,  50  were  cured. 

The  treatment  of  hernia  of  the  cord  must  not  be  expectant.  Before 
the  introduction  of  asepsis,  operation  was  shunned  and  the  treatment 
consisted  in  covering  the  sac  with  gauze;  with  small  hernia'  the  con- 
tents were  sometimes  reduced  and  the  hernial  sac  closed  with  plaster 
or  by  applying  silver  coins.  At  the  present  time  we  believe  that  every 
co-called  conservative  method  should  be  discarded  and  as  soon  after 
birth  as  possible  one  of  the  operations  recommended  should  be  per- 
il—3 


34  THE    DISEASES   OF   CHILDREN 

formed.  The  so-called  radical  operation,  first  performed  by  Lindfors  in 
1882,  is  the  simplest;  the  hernial  sac  is  opened  and  cut  away,  the  skin 
margins  are  freshened  and  after  reposition  of  the  hernial  contents  the 
abdominal  wound  is  sutured.  Amniotic  adhesions  must  be  loosened. 
Olshausen  recommends  an  extraperitoneal  method  in  which  the  amnion 
is  separated  from  the  peritoneum  and  the  latter  with  the  hernial  con- 
tents is  replaced  without  opening  the  peritoneal  cavity;  the  edges  of 
the  opening  are  then  freshened  and  sewed  together.  In  the  cases  in 
wliich  reposition  without  opening  the  sac  can  be  carried  out,  or  where 
the  contents  are  very  small,  C.  Breus  recommends  the  employment  of 
percutaneous  ligahire.  The  organs  are  replaced,  a  clamp  is  placed  around 
the  sac,  taking  in  the  skin,  and  the  sac  is  then  opened  and  dissected 
away;  below  the  clamp  two  or  more  sutures  are  passed  through  and 
through  and  knotted  and  the  clamp  removed. 

4.  Persistence  of  the  Ductus  Omphalomesentericus  (Vitelline  Duct). 
— Physiologically  the  omphalo-enteric  duct  wlaich  leads  from  the  yolk- 
sac  to  the  intestinal  tube,  is  obliterated  by  the  end  of  the  .second  fa?tal 
month.  In  case  obliteration  does  not  occur,  various  anomalies  can 
arise,  the  most  important  of  which,  for  the  newborn,  is  the  open  Meckel's 
diverticulum.  In  such  cases  the  ductus  omphalo-entericus  is  patent  in 
its  abdominal  portion  and  sometimes  for  a  short  distance  in  the  cord. 
After  the  cord  separates,  the  wound  does  not  heal  completely  and  there 
remains  a  narrow,  constantly  secreting  fistula.  In  marked  eases  the 
fistula  is  wide  open,  persistently  discharging  a  cloudy  fluid,  easily  iden- 
tified as  intestinal  contents.  In  other  cases,  fluid  may  be  obtained  by 
passing  a  small  soft  catheter;  examination,  chemical  and  microscopic, 
of  this  fluid  shows  it  to  be  intestinal  contents  (fat  globules,  acidity, 
odor,  negative  murexide  test). 

In  other  cases  the  umbilical  wound  heals  over,  but  a  short  time 
later  some  secretion  appears.  In  these  cases  epithelial  adhesions  close 
the  peripheral  end  of  the  duct  and  later  gi-adually  loosen.  In  other 
cases  a  tumor  about  the  size  of  a  hazel-nut  with  velvety  surface  is  found 
in  the  navel  region  either  immediately  after  the  separation  of  the  cord 
or  else  a  few  days  or  weeks  later;  tliis  shows  a  prolapse  of  the  wall  of 
the  fistula  and  in  rare  cases  a  prolapse  of  intestinal  coils  through  the 
fistula  can  occur.  Cases  of  umbilical  fistula'  in  wMch  the  walls  show  a 
structure  similar  to  the  gastric  mucosa  are  totally  obscure. 

The  diagnosis  of  an  open  Meckel's  diverticulum  is  as  a  rule  not  diffi- 
cult. It  is  important  not  to  confuse  the  small  tumor  resulting  from  pro- 
lapse of  the  mucosa  of  the  diverticulum,  with  a  sarcomphalus  or  an 
enteroteratoma.  It  can  be  differentiated  from  patent  urachus  fistula^ 
by  a  chemical  and  microscopic  examination  of  the  secretion. 

The  prognosis  is  very  good.  Rational  therapy  demands  the  excision 
of   the    whole    diverticulum    by   laparotomy.      However,    conservative 


DISEASES   OF   THE    NEWBORN 


3.5 


treatment  by  cauterizing  the  fistula  with  silver  nitrate  or  the  actual 
cautery,  sometimes  effects  the  closure  of  the  fistula. 

5.  Uraclnis  Fistidce. — The  stalk  of  the  allantois,  which  extends  to 
the  summit  of  the  bladder,  becomes  obliterated  in  its  extra-abdominal 
portion  during  the  second  foetal  month.  Its  abdominal  portion  per- 
sists as  the  urachus  and  its  lumen  is  either  totally  or  partially  nliliterated. 

Fig.  6. 


Persistence  of  the  ductus  omphalomesentericus.     Prolapsed  Meckel's  diverticulum. 

In  exceptional  instances  when  there  is  obstruction  to  the  outflow  of 
urine  through  the  urethra,  a  condition  occurring  more  often  in  boys 
than  in  girls,  the  lu-achus  remains  patent  throughout,  forming  a  fistu- 
lous tract  which  terminates  at  the  umbiHcus.  After  the  separation  of 
the  cord  one  finds,  at  the  umbilicus,  a  fistula  from  which  urine  is  passed, 
either  upon  pressure  or  during  micturition. 

In  some  cases,  just  as  with  a  patent  Meckel's  diverticulum,  a  small, 


36  THE   DISEASES   OF   CHILDREN 

tender,  red  tumor  is  found  in  the  umbilical  region,  varying  in  size  and 
bearing  the  fistulous  opening  on  its  summit.  The  tumor  owes  its  origin 
to  a  prolapse  of  the  mucosa  hning  the  urachus.  Probing  and  the  chemi- 
cal examination  of  the  evacuated  fluid  for  uric  acid,  as  well  as  its  micro- 
scopic examination,  confirm  the   diagnosis. 

The  treatment  consists  in  the  cauterization  of  the  fistulous  open- 
ing. Should  this  not  avail,  suture  of  the  walls  of  the  fistula,  after 
freshening  their  surface,  is  recommended. 

C.    DISEASES  OF  THE  NAVEL 

1.  AfYections  of  the  umbilical  cord  sometimes  occur  while  the  cord  is 
still  attached  to  the  child.  Moist  gangrene  (sphacelus)  occurs  very  com- 
monly. Instead  of  mummification  progressing  steadily,  the  stump  of 
the  cord,  either  wholly  or  in  its  central  part  alone,  becomes  discolored, 
swollen,  foul  smelling  and  a  brownish  secretion  soaks  through  the  dress- 
ings. Occasionally  a  small  mummified  portion  remains  behind  after 
the  separation  of  the  cord;  and  the  stumps  of  the  umbilical  vessels  are 
sometimes  seen  sticking  up  through  the  ragged  remains  of  the  cord.  In 
the  course  of  a  few  days  this  tissue  is  cast  off  by  means  of  a  serous  or 
seropurulent  inflammatory  demarcation.  These  disturbances  of  the 
separation  of  the  cord  are  often  accompanied  by  slight  fever,  a  symptom 
easily  understood,  since  bacteria  thrive  in  the  stump  of  the  cord  even 
under  normal  conditions. 

All  procedures  wliich  inliibit  the  normal  dessication  of  the  cord 
further  the  development  of  its  diseases.  Moist  heat  plays  the  most 
prominent  role  in  tliis  connection.  It  has  been  maintained,  but  not  at 
all  accepted,  that  the  daily  bath  of  the  infant  exerts  a  retarding  effect 
on  the  dessication  of  the  cord. 

The  prognosis  of  these  disturbances  is,  in  general,  favorable,  al- 
though the  infection  occasionally  spreads  and  can  then  lead  to  any  one 
of  the  various  diseases  of  the  umbilicus  itself.  Prophylactically  the 
strictest  asepsis  in  the  care  of  the  cord  and  the  avoidance  of  oily  or 
moist  dressings  are  enjoined. 

The  treatment  consists  in  removal  of  the  infected  cord,  preferably 
by  thermo-  or  galvano-cautery,  and  the  application  of  antiseptic  powders. 

2.  After  the  cord  separates,  a  serous  or  seropurulent  discharge 
sometimes  arises  and  persists  for  several  days;  this  secretion  is  usually 
caused  by  some  infectious  agent  but  in  rare  instances  may  be  the  result 
of  mechanical  or  chemical  irritation.  The  umbihcal  fold  is  either  nor- 
mal in  appearance  or  becomes  slightly  reddened  and  at  times  ocdema- 
tous;  after  wiping  away  the  discharge  flabby  gi-anulations  are  seen  at 
the  base  of  the  as  yet  unhealed  navel  wound.  This  condition  has  been 
termed  catarrhal  omphalitis,  excoriatio  umbilici  or  blennorrhoea  umbilici. 

The   diagnosis  should   be   made   only  when   the   other   conditions 


PLATE  6. 


DISEASES   OF   THE   NEWBORN  37 

which  give  rise  to  the  more  refractory  discharges  from  the  navel  can  be 
excluded,  such  as  diseases  of  the  umbilical  vessels;  fungus,  ulcers  and 
umbilical  gangi'ene. 

The  prognosis  is  very  good,  since  the  condition  heals  in  a  very  few 
days.  The  treatment  requires  the  use  of  antiseptic  dusting  powders 
(dermatol,  xeroform,  airol,  and  salicylic  acid)  or  pencilling  with  silver 
nitrate  (1-2  per  cent.)  or  peroxide  of  hydrogen  (2-3  per  cent.);  the 
application  of  a  1-2  per  cent,  borated  vaseline  dressing  or  a  moist  dress- 
ing with  liquor  alum.  acet.  (P.  G.),  diluted  8  times  in  water  or  aqua 
plumbi  (P.  G.),  diluted  2-3  times. 

3.  Umbilical  Ulcer. — Suppurative  processes  as  a  result  of  infection, 
can  arise  from  ulcerations  in  the  umbilical  wound.  In  tliis  case  the  um- 
bilical folds  are  oedematous,  covered  with  crusts  or  a  purulent  secre- 
tion and  sometimes  also  reddened.  On  separating  the  navel  folds  a 
smeary  gray  or  gray-green  exudate  appears,  which  in  some  cases  can  be 
easily  wiped  off,  but  in  others  is  fibrinous  and  adherent.  After  remov- 
ing the  exudate  the  ulcer  readily  comes  to  \'iew.  The  ulcers  occasionally 
extend  to  the  cutaneous  covering  of  the  navel  and  then  attain  consid- 
erable proportions  (see  illustration);  however  they  usually  are  only 
about  the  size  of  a  pea.  The  stump  of  the  umbilical  artery  can  often  be 
seen  at  the  bottom  of  the  idcer,  as  the  arterial  wall  offers  a  greater  re- 
sistence  to  the  necrotic  process  than  do  the  other  tissues;  the  perivas- 
cular tissue  is  always  involved  in  the  inflammatory  process. 

Umbilical  ulcers  are,  as  a  rule,  caused  by  infection  ^\^th  one  of  the 
well-known  bacteria  of  inflammation.  In  rare  cases,  however,  a  fibrin- 
ous exudate  is  the  sign  of  true  diphtheria,  the  confirmation  of  which 
lies  in  a  bacteriological  examination. 

The  diagnosis  of  ulcer  is  made  by  inspection. 

The  prognosis  is  good;  extensive  tissue  destruction  is  to  be  feared 
only  in  marantic  children. 

In  case  the  ordinary  antiseptic  wound  treatment  does  not  avail, 
the  treatment  consists  in  cauterization  either  with  silver  nitrate  or  with 
the  thermo-  or  galvano-cautery  (Escherfch). 

4.  Acute  Umbilical  Cellulitis  (Omphalitis). — In  rare  cases,  shortly 
after  the  separation  of  the  cord,  while  there  is  still  some  discharge  from 
the  navel  wound,  there  occurs  a  reddening  and  oedema  of  the  navel  fold, 
rapidly  spreading  to  the  surrounding  skin  of  the  abdominal  wall  for 
several  centimetres  and  extending  downwards  even  to  the  pubes.  The 
superficial  veins  of  the  abdominal  wall  are  regularly  congested  and  at 
times  the  inflamed  lymphatics  are  seen  as  stripes  extending  from  the 
navel. 

Pus  wells  up  from  the  bottom  of  the  umbilicus  unless  prevented 
by  crusts.  The  navel  often  protrudes  conically  and  the  skin  of  the 
navel  fold  and  vicinity  becomes  shiny  by  reason  of  the  tense  oedema; 


38  THE   DISEASES   OF   CHILDREN 

sometimes  the  whole  neighborhood  takes  on  a  bluish  tint  in  consequence 
of  the  venous  stasis. 

Fever,  restlessness,  excessive  whimpering,  distention  of  the  abdo- 
men and  anorexia  are  present.  The  legs  are  often  flexed  on  tlie  abdo- 
men. This  condition  consists  in  an  inflammation  of  the  subcutaneous 
tissues  of  the  umbilical  region. 

In  many  cases  abscesses  form;  in  a  smaller  number  gradual  retro- 
gression of  the  inflammatory  signs  take  place  and  in  rare  instances  com- 
plications like  gangrene,  arteritis,  phlebitis  and  peritonitis  occur.  One 
case  reported  by  Widerhofer  led  to  the  formation  of  an  intestinal  fis- 
tula. Acute  umbilical  cellulitis  has  its  origin  in  infection  of  the  umlnli- 
cal  wound  and,  therefore,  the  navel  itself  is  involved,  usually  the  seat 
of  an  ulcer  covered  with  a  lardy,  fibrinous  exudate. 

The  diagnosis  is  easily  made  by  reason  of  the  tense  oedema  and 
redness. 

The  treatment  consists  in  moist  compresses  of  liquor  alum.  acet. 
(P.  G.),  diluted  about  eight  times,  or  aqua  plumbi  (P.  G.),  diluted  twice, 
together  with  tlie  opening,  along  a  grooved  director,  of  any  abscesses 
that  may  be  ]5resent. 

5.  Umbilical  Arteritis  and  Phlebitis. — Infections  of  the  umbilical 
cord  and  umbilical  wound  very  often  lead  to  diseases  of  the  navel 
region,  which  spread  along  the  umbilical  vessels. 

These  infections  can  spread  in  two  ways.  In  the  first  place  they 
may  attack  Wharton's  jelly,  the  sheath  of  the  umbilical  vessels,  which 
extends  around  the  arteries  and  the  vein,  and  for  a  short  distance 
inside  the  abdomen;  then  the  infection  spreads  downwards  by  way  of 
the  lymphatics;  so  that  there  exists  a  periarteritis  or  periplilebitis  and 
from  this  vascular  disease  and  infection  of  the  thrombi  in  the  vessels 
can  arise.  The  second  way  is  by  direct  infection  of  the  vessels  and  then 
we  have  a  primary  arteritis  or  phlebitis.  The  second  way  is,  at  least 
for  the  artery,  the  less  freciuent,  the  disease  usually  beginning  as  a 
periarteritis. 

Infection  of  the  arteries  and  their  adventitia  (for  the  jelly  of  Whar- 
ton is  thus  to  be  considered),  is  much  more  frequent  and  also  much 
more  benign  than  infection  of  the  vein.  This  long  disputed  observa- 
tion, advanced  by  Bednar  and  by  Widerhofer,  stands  as  valid. 

Individual  cases  of  arteritis  and  periarteritis  show  marked  varia- 
tions in  their  course.  In  some  cases  the  disease  runs  its  course  without 
symptoms  and  can  go  on  to  recovery  without  ha\'ing  given  any  sign  of 
its  existence.  Umbilical  arteritis'  and  periarteritis  can  involve  either 
the  entire  length  of  the  vessel  or  only  a  portion  of  it.  In  cases  where 
only  a  portion  of  the  vessel  is  involved,  no  local  symptoms  occur  unless 
the  peripheral  portion  of  the  vessel  is  affected  as  far  as  the  navel.  In 
such  cases  the  navel  wound  is  covered  with  crusts  or  else  a  little  pus 


DISEASES   OF   THE    NEWBORN  39 

oozes  out  of  the  navel  ring  or  can  be  squeezed  out  by  stroking  the  ab- 
dominal wall  from  the  symphysis  toward  the  umbilicus.  Careful  inspec- 
tion will  occasionally  reveal  a  small  fistulous  opening,  often  passable 
ior  a  thin  probe  (with  great  care  on  account  of  the  danger  of  haemor- 
rhage in  case  the  sound  enter  the  lumen  of  the  vessel  and  dislodge  a 
clot).  The  probe  takes  a  direction  toward  the  sacrum  and  can  pass  for 
a  considerable  distance  downwards;  this  manoeuvre  should  alwaj's  be 
executed  without  any  force.  It  has  been  shown  that  the  probe  usually 
penetrates  the  necrotic  periarterial  tissue.  However,  in  other  instances 
of  circumscribed  uml)ihcal  arteritis  and  periarteritis,  the  peripheral  part 
of  the  vessel  is  perfectly  normal  and  the  navel  shows  no  visible  changes; 
but  a  portion  of  tlie  vessel  wall,  at  a  distance  from  the  end,  is  diseased. 
Inflammatory  processes,  with  or  without  abscess  formation,  arise;  the 
existence  of  these  inflammations  is  sometimes  manifested  only  by  a 
slight  fever,  in  the  majority  of  cases,  however,  they  run  their  course 
without  giving  rise  to  any  symptoms,  or  they  may  be  suspected  because 
of  failure  to  increase  in  weight  or  of  a  loss  in  weight  or  conditions  of 
collapse  in  the  child,  otherwise  inexplicable.  It  may  be  helpful,  then, 
for  diagnostic  purposes,  to  palpate  if  possiljle  the  thickened,  cord-like 
navel  arteries,  as  hard  resistances  through  tlie  abdominal  wall.  In  these 
cases  abscesses  form  between  the  peritoneum  and  the  abdominal  mus- 
cles and  extend  to  the  peritoneum  and  also  toward  the  scrotum,  where, 
burrowing  in  the  subcutaneous  cellular  tissue  they  may  reach  down  to 
the  tliigli. 

In  the  severest  cases  of  periarteritis  and  arteritis,  the  whole  vessel 
is  diseased  and  tlien,  besides  the  suppuration  at  the  navel  (wliich,  how- 
ever, may  heal  very  quickly)  there  may  occur  frequent,  though  slight, 
rises  of  temperature,  distention  of  the  abdomen,  dyspeptic  symptoms 
from  the  gastro-intestinal  tract,  not  uncommonly  purulent  peritonitis 
umbilical  haemorrhages  (Bednar)  and  also  septicemia  with  all  of  its 
severe  symptoms,  as  they  will  be  descriljed  in  the  follo\\-ing  section.  Be- 
sides peritonitis,  purulent  or  serous  pleurisy,  pneumonia,  subcutaneous 
abscesses,  phlegmons,  periostitis,  osteomyeUtis,  infarcts  in  the  spleen 
and  kidneys,  nephritis,  purulent  meningitis,  cerebral  haemorrhages,  and 
encephalitis  may  result. 

In  accord  with  the  researches  of  Basch  and  the  observations  of 
Finkelstein  we  are  justified  in  assuming  that  arteritis  and  periarteritis 
as  a  rule  remain  local  and  only  lead  to  systemic  infection  in  a  small 
percentage  of  cases. 

Doubtless,  however,  the  existence  of  umbilical  arteritis  through 
toxaemia,  lowers  the  resistance  of  the  child  and  thus  renders  him  more 
susceptible  to  other  maladies,  such  as  gastro-intestinal  afi'ections  and 
pneumonia,  although  these  latter  are  not  necessarily  to  be  considered  as 
evidence  of  a  general  septic  infection.    For  this  assumption,  the  identifi- 


40  THE   DISEASES   OF   CHILDREN 

cation  of  the  organisms  in  the  lungs  with  those  in  the  umbilical  vessels 
is  wanting,  as  is  also  their  demonstration  in  the  blood.  The  few  investi- 
gations of  this  nature  conducted  bj'  Finkelstein  gave  negative  results. 
It  is  important  to  know  that  infection  of  the  umbilical  vessels  can,  and 
in  most  instances  does  exist  without  any  visible  change  in  the  umbilicus 
itself.  It  is  just  the  very  severe  infections  that  are  especially  prone  to 
occur  while  the  umbiHcal  cord  is  still  attached  to  the  umbilicus.  There 
is  sometimes,  then,  a  delay  in  the  separation  of  the  cord  and  sometimes 
the  cord  shows  .slight  signs  of  putrefaction.  After  separation  of  the  cord, 
infection  of  the  vessels  or  their  sheaths  can  occur  primarily  without  in- 
volvement of  the  navel  or  secondarily  by  extension  from  a  local  disease 
of  the  umbilicus.  Tire  possibility  cannot  be  excluded,  however,  that 
umbilical  periarteritis  can  in  occasional  very  rare  instances  occur  as  a 
secondary  local  manifestation  of  septicEemia  arising  from  some  other 
cause. 

An  infection  of  the  umbilical  vessels  may  occur  any  time  after  the 
first  day  of  life  as  long  as  the  navel  wound  has  not  completely  healed; 
according  to  Buhl  it  can  be  acquired  even  in  utero  or  during  the  passage 
through  the  birth  canal.  The  infection  of  the  navel  probably  occurs 
ordinarily  from  the  fingers  of  the  nurse  or  physician,  possibly  through 
the  bath  or  dressings. 

The  frequent  coincidence  of  umbilical  disease  and  ophthalmia  sug- 
gests the  possibility  of  the  infection  being  carried  from  the  conjunctival 
pus  to  the  cord  (Runge).  Contamination  of  the  umbilical  wound  with 
infected  lochial  secretion  also  deserves  mention. 

The  micro-organisms  to  be  considered  as  cau.sing  arteritis  and  peri- 
arteritis are:  staphylococcus  pyogenes  aureus  and  albus,  bacterium 
coli,  streptococci,  bacillus  pyocyaneus  and  the  diplococcus  pneumoniae. 

The  disease  occasionally  occurs  epidemically  in  maternities  (Wasser- 
mann:  pyocyaneus  infections). 

The  duration  of  arteritis  and  periarteritis  varies  from  a  few  days 
to  several  weeks.  Premature  children  are  remarkably  prone  to  affections 
of  the  umbilical  vessels. 

The  autopsy  findings  in  severe  cases  of  arteritis  and  periarteritis 
show  extensive  inflammatory  lesions  in  the  vessels  and  perivascular 
tissues,— in  the  latter  alone,  in  a  few  early  cases.  The  pathological 
changes  involve  either  the  entire  vessel,  or  only  a  portion  of  it,  or  only 
the  perivascular  tissues.  Correspondingly  we  find  the  umbilical  arter- 
ies, which  are  usually  involved  on  both  sides,  thickened  and  cord-Uke; 
the  vessels  are  either  symmetrically  dilated  or  sacculated;  on  cross- 
section  the  walls  appear  considerably  thickened,  oedematous,  infiltrated, 
the  intima  lustreless  and  damaged  in  spots,  and  the  surface  of  the  vessel 
uneven.  In  the  lumen  there  are  sometimes  cheesy  masses,  sometimes 
pus  with  or  without  blood-clots.     In  severe  cases  the  changes  due  to 


DISEASES    OF    THE    NEWBORN  41 

septicgemia  or  caused  b}^  a  complicating  disease  are  never  lacking.  Not 
even  in  the  severest  cases  can  the  changes  in  the  vessels  be  traced  as 
far  as  the  retroperitoneal  tissue.  In  other  cases  the  intima  is  not  appre- 
ciably changed  and  the  vessel  contains  no  pus,  but  the  perivascular 
tissue  is  necrotic  over  a  large  area  and  converted  into  a  large  pus  cavity 
with  or  without  an  external  fistula. 

The  prognosis  of  the  disease  is,  in  general,  not  unfavorable;  cases 
running  a  prolonged  course,  however,  terminate  fatally  from  sepsis, 
peritonitis,  or  sometimes  from  a  complicating  pneumonia  or  nutritional 
disorder. 

Umbilical  phlebitis  and  periphlebitis  are  encountered  considerably 
less  often  than  arteritis  and  most  frequently  in  feeble  children.  The 
arteries  are  surrounded  by  a  much  stronger,  circular  layer  of  connec- 
tive tissue  (Runge)  which  accompanies  them  for  a  distance  of  a  few  cen- 
timetres inside  the  abdomen  and  this  furnishes  a  good  culture  medium 
for  infections.  But  there  is  only  a  scanty  layer  of  Wharton's  jelly 
around  the  vein  and  this  extends  for  only  a  short  distance.  The  rarity 
of  umbilical  phlebitis  depends  upon  these  anatomical  relations;  more- 
over, it  is  usually  accompanied  by  an  arteritis.  In  a  large  number  of 
cases,  umbilical  phlebitis  gives  rise  to  no  local  signs  at  the  umbilicus. 
Thus  the  dictum  of  Porak  and  Durante  seems  Justified,  that  the  severest 
cases  of  umbilical  infection  show  the  slightest  local  signs.  The  exis- 
tence of  a  phlebitis  can  only  be  assumed  clinically,  from  the  various 
general  symptoms  of  septicemia  to  wliich  the  phlebitis  regularly  leads. 
According  to  Bednar,  pus  cannot  be  expressed  from  the  vein  in  uncom- 
plicated cases;  in  such  cases  diagnostic  importance  must  be  ascribed 
to  a  gi-adual  daily  increase  in  the  intensity  of  the  icterus  up  to  a  dark 
discoloration  of  the  skin  (ictere  bronze,  Porak  and  Durante),  possibly 
also  to  an  oozing  of  blood  from  the  navel  while  in  the  process  of  healing. 
Other  signs  of  sepsis,  namely,  those  on  the  serous  membranes,  are  like- 
wise usually  not  primary  in  the  newborn,  but  secondary,  and  may  then 
have  diagnostic  value. 

Sometimes  such  conjplications  as  erysipelas,  umbilical  gangrene,  etc., 
arise,  and  expedite  the  fatal  outcome,  which  practically  always  super- 
venes. 

At  autopsy  one  often  finds  pus  in  the  vein,  sometimes  only  a  tliick 
coagulum  which  fills  the  greatly  dilated  vein  (this  of  itself  is  patholog- 
ical), and  is  infected  by  bacteria  (Porak  and  Durante);  sometimes  diffuse 
hepatitis  or  else  multiple  abscesses  in  the  liver;  oedema  or  inflammation 
of  the  capsule  of  Glisson;  very  often  peritonitis;  and  other  signs  of 
sep.sis. 

Whereas  the  prognosis  of  umbilical  arteritis  umbilicalis  is  compar- 
atively favorable,  that  of  umbilical  plilebitis  is  very  unfavorable,  as  the 
malady  is  almost  ine\'itably  fatal. 


42  THE   DISEASES   OF   CHILDREN 

The  prophylaxis  of  all  navel  affections  demands  strict  asepsis  in 
the  care  of  the  navel  and  the  employment  of  methods  which  favor  the 
mummification  of  the  cord. 

The  treatment  of  this  group  of  umbilical  diseases  avails  practically 
but  little;  surgical  intervention  can  only  be  recommended  when  there 
are  circumscribed  abscesses  in  the  first  portion  of  the  artery  or  in  the 
periarterial  tissue.  Otherwise  we  limit  ourselves  to  the  employment  of 
antiseptic  umbilical  dressings  with  antiseptic  powders  or  wet  dressings 
with  hquor  alum.  acet.  (P.  G.),  diluted  8-10  times.  These  may  be  ap- 
plied with  the  "apron  dressing"  of  Flick,  which  Escherich  recommends. 
Stimulants  must  be  given  in  conditions  of  collapse  and  with  complica- 
tions the  appropriate  local  or  symptomatic  treatment  must  be  insti- 
tuted. 

6.  Umbilical  Gangrene. — Since  the  recognition  of  asepsis  gangi-ene 
of  the  navel,  which  was  formerly  very  frequent,  has  become  one  of  the 
rarest  diseases,  occurring  only  in  feeble  or  poorly  nourished  children  in 
the  first  weeks  of  life  (according  to  Bednar  exceptionally  as  late  as  the 
9th  week).  It  usually  follows  an  omphalitis  or  some  other  local  navel 
affection;  more  rarely,  in  atrophic  children,  it  occurs  without  any 
demonstrable  local  disease.  The  destructive  process  involves  the  navel 
in  a  circular  area  of  greater  or  less  extent,  sometimes  with  and  sometimes 
without  the  formation  of  blebs;  it  next  attacks  the  muscular  layers, 
causes  peritonitis  and  sometimes  leads  to  perforation  of  the  peritoneum 
and  extrusion  of  the  viscera.  In  very  rare  cases  necrosis  of  the  wall  of 
an  intestinal  coil,  lying  adjacent  in  front,  occurs,  with  the  formation  of 
an  artificial  anus;  then  intestinal  contents  are  discharged  from  the 
navel;  in  case  the  intestinal  coil  adjacent  does  not  become  at  first  ad- 
herent, a  diffuse  peritonitis  will  arise  as  a  result  of  infection  of  the  peri- 
toneum by  feces.  Erosion  of  the  umbilical  vessels  and  severe  ha?mor- 
rhage  are  also  among  the  dangers  of  this  malady.  In  cases  of  extensive 
gangrene,  collapse  with  or  without  fever  and  death  in  coma  usually 
occur.  The  few  cases  .(according  to  Fiirth,  15  per  cent.)  in  which  recov- 
ery has  been  observed,  soon  show  a  cessation  of  the  necrosis;  round 
about  the  gangrenous  area  a  reactive  infiamniation  goes  on,  granula- 
tions are  formed  and  healing  takes  place. 

The  treatment  by  using  the  thermo-  or  electro-cautery  far  into  the 
healthy  tissue  (as  with  noma),  might  possibly  avail  in  the  beginning  of 
the  disease;  this  procedure  should  not  be  carried  out  in  very  atrophic 
children.  Otherwise,  antiseptic  powders,  cauterizing  with  nitrate  of 
silver  and  warm  baths  have  been  recommended. 

7.  Umbilical  Fungus. — (Sarcomphalus,  umbilical  granuloma). — As 
early  as  the  second  or  third  week  of  life,  a  tumor  may  form  within  the 
navel  fold  as  the  result  of  the  failure  of  the  navel  wound  to  heal  and  of 
the  consequent  abnormal  proliferation  of  the  granulations.    This  tumor, 


DISEASES    OF    THE    NEWBORN  43 

at  times  flat  and  sessile  and  at  times  pedunculated,  is  attached  to  the 
base  of  the  navel  wound,  is  fleshy  in  color,  has  an  irregular  raspberry- 
like surface  and  gradually  attains  the  size  of  a  pea  or  even  a  hazel-nut. 
At  times  the  tumor  is  hidden  by  the  folds  of  the  navel  and  can  be  seen 
only  when  the  folds  are  held  apart. 

In  case  these  formations  are  left  to  themselves,  an  eczema  of  the 
skin  of  the  navel  fold  arises  as  a  result  of  the  persistent  secretion;  the 
tumor  persists  for  weeks  and  even  months,  but  finally  shrinks  gradually 
and  becomes  covered  with  skin.  In  adult  life  one  occasionally  finds  in 
the  umbilicus,  little  projections  covered  with  skin,  representing  the 
remains  of  such  fungi. 

The  histological  examination  of  these  tumors  reveals  in  the  vast  ma- 
jority of  cases  a  highly  vascular  granulation  tissue  (Kiistner);  in  a  num- 
ber of  cases,  however,  the  tumor  is  composed  of  glandular  tubules,  lined 
with  c3dindrical  epithelium,  or  else  of  smooth-muscle  and-  connective 
tissue.  These  neoplasms  are  then  known  as  enteroteratomata  (Kolac- 
zek)  or  adenomata  (Kiistner)  and  owe  their  origin  to  partial  prolapise 
of  the  imperfectly  involuted  omphalo  mesenteric  (vitelline)  duct  (Ko- 
laczek),  or  else  to  a  proliferation  of  cells,  epithelial  in  nature,  which  are 
left  behind  in  the  granulation  tissue  at  the  time  of  the  separation  of  the 
cord  (v.  Hiittenl)renner).  In  a  few  cases  the  last-mentioned  author 
found  cuboid  epithehal  cells  (rests  from  the  urachus)  which  had  sub- 
sequently proliferated. 

The  diagnosis  is  easily  made.  One  must  only  bear  in  mind  the  possi- 
bihty  of  patent  urachus-fistula  with  a  protuberant  mucosa  or  a  persis- 
tent patent  vitelline  duct,  the  peripheral  end  of  which  is  somewhat  pro- 
lapsed. In  the  latter  case  intestinal  contents  issue  from  a  fistula  which 
admits  the  passage  of  a  probe  for  a  considerable  distance.  Moreover 
these  latter  swellings  have  a  tumor-like,  smooth  surface  and  are  not  un- 
even and  irregular.  Since  the  sarcomphalus  does  not  heal  spontaneouslj' 
or  else  very  slowly,  it  should  be  touched  with  kmar  caustic  to  hasten  its 
involution.  In  other  cases  it  can  be  tied  off  with  silk,  which  insures  its 
separation  after  2-3  days  or  it  can  be  cut  off  with  the  scissors,  after 
ligature.  All  these  manipulations  are  painless  since  the  sarcomphalus 
contains  no  nerve-endings. 

Other  tumors  of  the  umbilical  region  are  extremely  rare.  Angi- 
omata,  myxosarcomata  and  cystomata  have  been  reported. 

8.  Umbilical  HcEmorrhagca. — Hannorrhages  from  the  navel  may  be 
divided  into  three  classes.  The  first  group  comprises  the  cases  in  which 
bleeding  occurs,  only  from  the  arteries,  shortly  or  only  a  few  hom's  after 
birth,  despite  the  ligation  of  the  umbilical  vessels.  Tliis  haemorrhage 
comes  from  the  cut  surface,  of  the  cord  and  occurs  occasionally  imme- 
diately following  its  ligation  and  cutting.  This  can  occur  onlj-  under 
two  conditions:  first,  when  the  ligature  is  not  sufficiently  secm-e;  and 


44  THE    DISEASES   OF   CHILDREN 

second,  when  the  physiological  cessation  of  the  circulation  in  the  um- 
bilical arteries  does  not  take  place.  Shortly  after  birth  the  umbilical 
arteries  are  normally  no  longer  filled  by  the  blood  current,  since  they  are 
shunted  out  of  the  arterial  circulation  as  a  result  of  the  expansion  of 
the  lungs  and  the  fall  in  blood  pressure.  Moreover,  the  ligation  of  the 
cord  is  followed  by  contraction  of  the  muscular  coat  of  the  umbilical 
arteries  and  closure  of  the  vessels. 

The  umbilical  artery  is  well  adapted  for  this  powerful  closure  by 
reason  of  the  peculiar  arrangement  of  its  musculature,  as  described  in  a 
previous  chapter  (Preliminary  Physiological  Remarks).  Normally,  there- 
fore, ligation  of  the  cord  would  be  supererogatory.  However  there  are 
children  in  whom  the  fall  of  the  blood  pressure  fails  to  take  place  and 
in  whom  death  from  haemorrhage  might  occur,  should  the  cord  not  be 
ligated.  To  this  category  belong  the  asphyctic  newborn  infants;  or  those 
whose  lungs  have  only  partially  expanded;  especially  also,  premature 
children;  also  those  who  for  other  reasons  have  been  partially  asphyx- 
iated and  finally  children  in  whom  abnormal  conditions  of  the  blood 
pressure  prevail  on  account  of  disturbances  of  the  circulation  (congeni- 
tal heart  disease).  E.  Hofmann  calls  attention  to  the  fact  that  suffo- 
cation itself  causes  a  rise  in  the  arterial  pressure  which  would  facilitate 
umbilical  hjemorrhage. 

In  some  cases  the  bleeding  does  not  occur  immediately  after  the 
cutting  of  the  cord  but  follows  some  hours  later.  In  these  cases  the 
ligature  is  applied  correctly  but  as  a  result  of  the  drying  up  and 
shrinkage  of  the  cord  the  band  becomes  loose  and,  if  the  vessels 
have  not  contracted,  they  bleed.  The  above-mentioned  forms  must 
be  designated  umbilical  cord  htemorrhages  in  contradistinction  to 
the   following. 

The  second  group  comprises  those  rare  cases  in  which  the  htemor- 
rhage  occurs  from  the  lumen  of  the  umbilical  vessels  (and  here  moreover 
it  is  exclusively  the  arteries  and  not  the  veins  which  bleed)  at  the  level 
of  the  umbilicus.  Ritter,  who  was  able  to  report  97  cases  of  umbilical 
haemorrhage  saw  this  bleeding  from  the  vessels  only  seven  times. 
The  blood  came  in  spurts  in  one  case  only;  in  the  others  in  small  drops. 
In  Grandidier's  collection  of  202  cases  there  are  only  7  cases  of  bleeding 
from  the  vessels,  and  among  these,  two  cases  in  which  the  blood  spurted 
from  the  vessels. 

This  haemorrhage  occurs  within  the  first  days  of  life  (according  to 
Ritter,  at  the  latest  on  the  15th  day).  The  cord  has  always  separated 
or  else  is  in  the  process  of  separation,  since  it  clings  longest  to  the  ar- 
teries and  these  (or  only  one  of  them)  must  be  separated  before  a  lurm- 
orrhage  from  the  vessel  can  occur  at  the  level  of  the  umbilicus.  In  con- 
nection with  this,  the  normal  closure  of  the  intra-abdominal  portion  of 
the  navel  vessels  must  have  failed. 


DISEASES   OF   THE    NEWBORN  45 

The  parenchymatous  umbilical  hcBmorrhages  which  form  the  third 
group  are  relatively  the  most  frequent.  With  this  condition,  although 
the  umbilical  vessels  are  closed,  the  blood  trickles  "just  as  from  a 
sponge"  from  the  small  vessels  and  from  the  capillaries  at  the  base  of 
the  navel,  both  before  and  after  the  separation  of  the  cord.  This  bleed- 
ing usually  occurs  in  the  first  or  second  week  of  life  and  only  in  rare 
cases  and  then  in  poorl}'  nom'ished  infants,  does  it  come  on  later  (Ritter 
reports  one  case  occurring  on  the  63rd  day  of  life).  The  bleeding  either 
takes  place  continuously  or  interruptedly,  it  lasts  many  hours  and  even 
daj's  and  leads  to  serious  loss  of  blood  which  often  causes  the  death  of 
the  child.  According  to  Grandidier  only  17  per  cent,  of  these  children 
recover.  This  depends  only  partly  on  the  fact  that  the  children  bleed 
to  death,  in  part,  however,  the  children  die  of  the  underlj-ing  consti- 
tutional disease. 

Parenchymatous  umbilical  haemorrhage,  like  haemorrhage  from  the 
umbilical  vessels,  is  in  the  majority  of  cases  the  result  of  a  demonstraljle 
sepsis  and  is  often  (but  not  always)  accompanied  by  symptoms  of  that 
disease.  Haemorrhages  from  other  organs  and  from  parts  other  than  the 
navel  often  occur  T\Tth  this  condition,  and,  not  seldom,  local  diseases  of 
the  navel  [arteritis-phlebitis,  umbilical  gangi-ene  (Ritter)]. 

In  other  cases  umbilical  ha?morrhage  is  referable  to  hereditary 
syphilitic  disease  of  the  vessels.  This  must  be  admitted  as  etiologically 
of  moment  (reference  to  tliis  subject  will  be  found  in  the  chapter  on 
Mekena),  since  Bondi  only  recently  has  again  described  specific  changes 
in  the  blood  vessels  due  to  hereditary  syphilis. 

It  does  not  seem  to  have  been  demonstrated  that  umbihcal  haemor- 
rhage ever  occurred  as  the  result  of  hacmophiha.  One  finds,  however, 
in  Grandidier's  tables  a  few  cases  of  such  ha?morrhage  occurring  in  chil- 
dren whose  mothers  belong  to  haemophihc  famiUes,  but  in  these  cases 
other  cases  of  navel  bleeding  do  not  seem  to  be  excluded.  (The  eti- 
ology of  local  haemorrhage  in  the  newborn  receives  consideration  in  the 
chapter  on  Meltena.)  W^e  do  not  consider  as  pathological  the  shght 
bleeding  wliich  takes  place  on  the  days  immediately  following  the  sepa- 
ration of  the  cord  or  which  occurs  from  the  mechanical  irritation  of  the 
granulations  (e.g.  from  the  examination  by  the  physician  or  from  rub- 
bing ^ith  cotton,  etc.).  Occasionally  traces  of  blood  or  small  crusts  are 
then  found  on  the  navel  dressings. 

Therapy. — Haemorrhage  from  the  cord  may  be  controlled  b}-  ener- 
getic Ugation,  possibly  wath  heavy  silk  or  \s-ith  rubber  bands  (drainage 
tube)  (Budin).  After  separation  of  the  cord  the  bleeding  can  be  stopped 
by  apphcations  of  adrenahn  solution,  or  ferric  chloride  or  calcium  phos- 
phate and  compression  and,  finally,  by  suture  of  the  umbihcus.  Should 
one  have  to  deal  ^\•ith  the  rare  instance  of  a  genuine  bleeder  (haemophilia) 
or  with  a  child  whose  blood  has  a  greatly  impaired  capacity  for  clotting, 


46  THE    DISEASES   OF   CHILDREN 

in  which  case,  as  with  the  septic  disease,  the  blood  oozes  from  the  needle- 
holes,  the  hiemorrhage  must  be  controlled  by  continuous  compres- 
sion. In  all  cases  of  umbilical  haemorrhage  wluch  cannot  be  controlled 
by  agents  applied  locally,  the  use  of  subcutaneous  injections  of  gela- 
tine (Merck's,  absolutely  sterile),  in  the  dose  of  10-20  c.c,  (2  to  6  drams) 
is  recommended. 

9.  Umbilical  Hernia. — (Navel  ring  hernia,  hernia  umbilicaUs). — 
Protrusion  of  the  abdominal  contents  through  the  navel  ring  occurs 
^^^th  great  frequency  in  childhood  and  usually  in  the  first  months  of 
life.  The  navel  ring,  especially  in  its  upper  section,  is  closed  only  by  a 
few  strands  of  connective  tissue.  The  place  which  corresponds  to  the 
passage  of  the  umbihcal  vein  through  the  navel  ring  and  which  is  called 
the  umbilical  canal  by  Herzog  (see  anatomical  introduction)  easily 
gives  way  to  the  intestinal  pressure  occurring  when  the  child  cries  and 
strains;  the  fascia  and  peritoneum  are  pushed  forward,  the  intestines 
enter  and  gradually  widen  the  aperture,  thus  causing  ruptures  from  the 
size  of  a  pea  up  to  that  of  a  prune.  Schmidt  assumes  that  a  pit-hke 
depression  must  be  found  in  the  peritoneum  by  the  time  the  navel  heals 
in  order  for  a  hernia  to  develop. 

Symptoms. — When  the  child  Hes  quietly  a  loose  pocket  of  skin  can 
be  seen  in  the  navel  region.  When  the  hernia  is  small,  it  is  covered  with 
normal  skin  and  when  larger  with  thinned-out  skin.  At  its  summit  or 
a  Uttle  below,  is  the  thinned  out  navel  scar.  When  the  child  strains  or 
cries,  the  intestinal  contents  are  pressed  forward  into  the  sac,  and  they 
can  be  reduced  with  a  gurgling  sound.  Then  the  sharply  defined  open- 
ing of  the  hernial  orifice  which  is  usually  round,  can  be  felt;  its  circum- 
ference is  formed  by  the  navel  ring.  The  hernial  sac  consists  of  the  peri- 
toneal protrusion  and  the  fascia  umbilicalis,  while  the  small  intestine 
and  rarely  the  omentum  form  its  contents. 

Course  and  Prognosis. — -Umbilical  heruise  in  nurslings  heal  spon- 
taneously in  the  majority  of  instances.  HeaUng  in  infancy  fails  in  only 
a  small  number  of  cases  and  then  the  probabiUty  of  healing  decreases 
from  year  to  year.  Umbilical  hernise  not  healed  within  the  first  3-4 
years  remain  open  for  life  unless  treated  by  operation.  In  some  cases 
adhesions  between  the  sac  and  contents  occur  but  strangulation  occurs 
very  rarely. 

Prophylaxis  and  Treatment. — Inasmuch  as  umbihcal  hernise  occur 
in  a  very  large  number  of  nurslings  during  the  first  months  of  life,  it  is 
incumbent  upon  the  physician  to  examine  the  umbilical  wound  care- 
fully, and  if  there  be  the  shghtest  sign  of  a  stretching  of  the  scar  and 
especially  if  the  baby  cries  a  great  deal  or  strains  as  the  result  of  consti- 
pation, it  is  well,  after  the  third  week  of  hfe,  to  apply  an  umbihcal 
bandage,  as  a  prophylactic.     The   wearing  of  a  navel  binder  is  futile. 

Adhesive  straps  answer  this  purpose  best.     Two  or  three  strips  of 


DISEASES    OF    THE    NEWBCMIX  47 

adhesive  plaster,  2-32  cm.  (I-I2  in.)  wide,  and  about  12  cm.  (5  in.) 
long,  are  used;  the  skin  in  the  navel  region  is  drawn,  b}'  the  mother  or 
an  assistant,  over  the  navel  into  two  parallel  linear  folds,  forming  an 
elastic  cushion  over  the  hernial  orifice;  the  straps  are  tightly  appUed 
overlapping  one  another  and  thus  effectually  hold  this  cushion  down. 
Instead  of  the  three  strips,  one  strip  about  50  cm.  (20  in.)  long  can  be 
used,  passing  it  around  the  body  and  crossing  it  over  the  skin-folds  held 
by  the  mother  or  assistant.  Many  physicians  recommend  the  use  of  a 
flat  disc,  for  example  a  gold  piece,  fastened  over  the  hernial  orifice  with 
adhesive  plaster.  The  adhesive  should  be,  as  far  as  possible,  non-irri- 
tating and  should  be  changed  at  least  once  a  week.  The  treatment  should 
be  interrupted  if  necessary  until  the  subsidence  of  any  eczema  that 
may  arise  from  the  irritation  of  the  strips. 

If  heahng  of  the  hernia  does  not  take  place  within  the  first  year, 
the  further  use  of  the  adhesive  bandage  is  usually  without  avail  and  then 
recourse  must  be  had  to  spring  or  elastic  trusses. 

Escherich  recently  recommended,  in  such  cases,  the  introduction 
of  an  intraperitoneal  paraffine  disk.  For  tliis  purpose  the  child  is 
auEesthetized  and  by  means  of  a  special  syringe,  a  few  c.c.  of  paraffine 
(melting  point  39°  C;  102°  F.),  are  injected,  into  the  hernial  sac,  pre- 
\iously  emptied  of  its  contents  by  lateral  compression.  The  paraffine 
is  then  hardened  by  means  of  an  ether  spray  directed  by  an  assistant, 
and  is  molded  by  pressure  into  the  form  of  a  disk.  The  point  of  punc- 
ture is  covered  with  sterile  gauze  and  an  adhesive  dressing  applied  as  in 
the  conservative  treatment.  In  other  cases,  those  in  older  children,  and 
where  the  hernial  orifice  is  larger,  an  operation,  consisting  of  the  extra- 
peritoneal reposition  of  the  hernial  sac  and  contents,  is  necessary.  Ven- 
tral hernia  can  be  prevented  by  dissecting  the  recti  from  their  sheaths 
and  sewing  their  edges  together. 

IV.     INFECTIOUS  DISEASES  OF  THE  NEWBORN 

A.     TETANUS  NEONATORUM 

Tetanus  neonatorum  is  classed  with,  the  wound  infections  of  the 
newborn,  just  as  erysipelas  of  the  newborn,  because  its  port  of  infection 
is  regularly  the  umbiUcal  wound.  Tetanus  in  the  newborn  runs  a  sim- 
ilar course  to  that  in  the  adult  and  differs  in  no  wise  from  the  latter  in 
etiolog}'  or  symptomatologj-.  The  healing  of  the  navel  wound  is  usually 
complete  in  about  14  days  and  tliis  implies  that  tetanus  neonatorum 
is  limited  to  the  first  three  weeks  of  life,  except  when  the  virus  gains 
entrance  elsewhere  than  at  the  navel. 

Symptoms. — The  disease  starts  shortly  after  birth,  usually  in  the 
second  week,  less  often  at  the  end  of  the  first  or  during  the  tliird  week 
of  life.     The  onset  is  accompanied  by  great  restlessness  and  frequent 


48 


THE   DISEASES   OF   CHILDREN 


piercing  cries.  It  soon  becomes  evident  that  the  child  has  some  diffi- 
culty in  sucking.  When  the  infant  is  put  to  the  breast  or  given  the 
bottle,  it  makes  only  one  or  at  the  most  a  few  sucking  movements  and 
then  desists.  The  examining  physician  at  tliis  time  finds  a  spasm  of 
the  muscles  of  mastication  (trismus)  which  is  ehcited  as  often  as  the 
child  is  induced  to  suck  and  which  later  becomes  permanent.  This 
tetanic  spasm,  the  principal  symptom  of  the  disease,  is  not,  however, 
long  limited  to  the  inasseters.  Within  a  few  days  or  even  hours  the 
recurring  attacks  of  tonic  convulsions  spread  to  the  muscles  of  the  face 
and  there\\ith  the  face  takes  on  a  characteristic  appearance  (risus  sar- 


FiG.  8. 


Fig.  7 


Tetanus  neonatorum. 
^Facies  tetanica.) 


Tetanus  neonatorum.  (Wrinkling 
of  tlie  skin  of  the  face  and  spasm  of  the 
upper  extremities.) 


donicus,  facies  tetanica).  The  forehead  is  gathered  into  deep  wTinkles, 
very  striking  for  a  child  of  tliis  age;  the  eyelids  are  squeezed  together, 
the  puckered  mouth  is  protruded  more  or  less;  at  times  the  corners  of 
the  mouth  are  drawn  sharply  downwards,  in  other  cases  the  upper  lip 
alone  is  contracted  or  else  to  a  greater  degree  than  the  lower  lip,  thus 
drawing  the  skin  here  into  radiating  folds.  The  spasm  next  extends  to 
the  muscles  of  the  neck  and  back;  the  head  is  strongly  retroflexed, 
opisthotonos  begins,  the  abdominal  walls  become  rigidily  contracted, 
the  upper  extremities  usually  rigidly  flexed  to  an  acute  angle  and  the 
legs  extended.     The  hands  are  usually  flexed  in  all  joints  and  the  feet 


DISEASES   OF   THE    NEWBORN 


49 


held  in  dorsal  flexion.  The  position  of  tlic  extremities  varies  according 
to  the  muscle  group  which  is  most  intensely  affected  by  the  tetanic 
convulsions. 

At  this  stage  on  account  of  the  spasm  of  the  masseters  and  the 
muscles  of  deglutition,  sucking  and  swallowing  become  impossible.  Not 
uncommonly  tonic  spasms  of  the  larynx  and  diaphragm  occur.  Emit- 
ting no  sounds,  the  child  hes  prostrate,  becomes  cyanotic  and  breathes 
irregularly  and  superficially.  The  attacks  become  more  frequent  and 
of  longer  duration  so  that  they  seem  to  be  almost  uninterrupted.  They 
are  aggravated  by  external  stimuU.  Touching  the  skin,  taking  hold  of 
the  nipple,  or  cooling  the  body  surface  by  uncovering  the  child  serve  to 
arouse  stormy,  jerky,  tetanic  contractions.  In  many  cases  the  disease 
runs  an  afebrile  course,  in  others  however  it  is  accompanied  by  irregu- 
lar fever  which  at  times  is  very  high.  A  post-mortem  ri.?e  in  the  body 
temperature  has  been  found  very 


^ 


Fig.  9. 

often  in  cases  of  tetanus.     The . 

complete  cUnical  picture  is  not 
alwaj's  encountered.  In  many 
cases  the  spasms  extend  no  fur- 
ther than  the  masseters,  accom- 
panied by  slight  contractions  of 
one  or  the  other  muscle  groups, 
usually  of  the  face  and  neck. 

In  the  further  course  of 
such  light  cases,  the  spasms 
gradually  diminish  in  frequency 
and  inten.sity  and  finall}'  cease 
entirely.  Even  fully  developed 
cases  of  tetanus  may  end  in  re- 
covery; the  spasms  diminish 
gradually,  especially  the  trismus 
and  spasms  of  deglutition;   then 

the  rigidity  diminishes,  and  convulsions  occur  only  after  powerful 
stimuU  and  later  not  at  all;  in  such  cases  I  have  observed,  even  after 
the  disappearance  of  the  tonic  spasms,  a  slow  contraction  of  the  mus- 
cles, elicited  by  the  tap  of  a  hammer,  which  persisted  quite  a  while. 

As  a  rule,  in  uncompHcated  cases  the  other  organs  show  nothing 
noteworthy.  It  must  be  noted,  however,  that  septic  infection  may 
often  accompany  tetanus  in  the  newborn.  The  navel  wound  presents 
either  nothing  abnormal,  or  else  exudes  a  serous,  sero-sanguinolent  or 
purulent  secretion.  In  many  cases  the  umbihcal  wound  has  a  lardy 
•exudate  and  has  the  characteristics  of  an  umbihcal  ulcer. 

Etiology. — Tetanus  neonatorum,  like  tetanus  in  the  adult,  is  due  to 
a  specific  agent,  the  tetanus  bacillus.     The  older  opinions  which  attrib- 
II— 4 


Tetanus  neonatorum. 


50  THE   DISEASES   OF   CHILDREN 

uted  tetanus  to  the  use  of  baths  of  too  high  temperature,  to  the  influ- 
ence of  drafts,  cold,  etc.,  must  be  regarded  as  false.  Nicolaier  in  1885 
discovered  and  cultivated  the  tetanus  bacillus  and  Kitasato  obtained  it 
in  pure  culture.  It  is  a  very  constant  inhabitant  of  the  ground,  in  gar- 
dens, and  ha.s  also  been  found  in  the  dust  of  dweUings,  in  cracks  in  the 
floors,  in  furniture,  etc.,  (Bilumler).  It  gains  entrance  to  the  umbili- 
cal wound  with  dust,  probably  by  contamination  from  the  baby-clothes, 
the  bandage,  or  the  hands  of  the  nurse.  There  may  perhaps  be  an  in- 
fection of  the  umbihcal  cord,  just  as  with  sepsis.  In  the  newborn,  the 
bacillus  of  tetanus  was  demonstrated  by  Kitasato,  in  a  case  of  Bagin- 
sky's  and  later  also  by  Peiper,  Baumler,  and  others.  The  bacillus  of 
tetanus  multipUes  at  the  seat  of  infection  by  spore  formation  and,  ex- 
cept in  the  rarest  instances,  does  not  invade  the  body;  it  produces  toxins 
however,  which,  according  to  the  investigations  of  H.  Meyer  and  Ransom, 
are  absorbed  from  the  lymph-spaces,  for  the  most  part  through  the  ends 
of  the  motor  nerves,  and  probably  through  the  axis-cyUnders  of  the 
motor  nerves.  These  toxins  reach  the  central  nervous  system,  where 
they  become  fixed.  The  combination  of  these  toxins  with  the  motor 
cells  in  the  anterior  horns  of  the  spinal  cord  and  the  nuclei  in  the  medulla, 
gives  rise  to  an  abnormal  increase  in  the  irritability  of  these  centres  and 
therein  hes  the  cause  of  the  tetanic  contractions.  The  motor  centres 
then  react  to  very  insignificant  stimuli.  The  peripheral  nerves  and  the 
muscles  are  not  involved,  as  is  proven  by  the  cessation  of  the  tetanic 
spasms  after  section  of  the  nerve-trunks. 

Before  the  onsent  of  the  tetanus  there  is  an  incubation  period  vary- 
ing from  two  days  to  a  few  weeks  after  the  infection  with  the  bacillus 
tetanus.  This  fact  however  cannot  have  any  relation  to  the  time  needed 
for  the  production  of  a  quantity  of  poison  sufficient  to  produce  its  effect, 
but  probably  depends  upon  the  fact  that  the  passage  of  the  poison 
through  the  nerve-trunks  requires  a  certain  time  before  it  reaches 
the  motor  centres.  Others  have  assumed  that  the  irritation  of  the 
motor  centres  is  not  due  to  the  toxins  per  se,  but  to  a  compound  of  the 
toxins  and  body-substances.  These  poisonous  compounds  are  sup- 
posed to  circulate  in  the  blood  of  the  inthvidual  infected  with  tetanus 
and  the  blood  of  such  individuals  should  therefore  immediately,  with- 
out a  latent  period,  produce  tetanic  spasms  in  the  mouse,  an  animal 
especially  susceptible  to  tetanus.  In  many  cases  the  tetanus  toxin  also 
circulates  in  considerable  quantities  in  the  blood  of  infected  individuals. 
In  such  cases  experimental  injection  of  the  toxin-containing  blood  may 
produce  tetanus.  TMs  has  not  only  been  estabhshed  as  regards  the 
blood  of  adults  and  of  animals,  but  also  in  one  case  of  tetanus  neo- 
natorum. 

Even  at  the  very  beginning  of  the  disease  the  tetanus  bacillus  is 
still  demonstrable  in  the  navel  wound  in  only  a  small  number  of  the 


DISEASES    OF    THE    NEWBORN  51 

cases.  It  prdhiilily  dies  off  very  (juickly.  Other  septic  bacteria  are 
constantly  found  here. 

Escherich  recommends  curetting  the  navel  wound  wilii  a  sharp 
spoon  in  order  to  demonstrate  the  bacillus.  The  scrapings  should  be 
used  for  the  inoculating  of  mice  and  making  cultures. 

Duration  and  Course.— Tetanus  neonatorum  runs  a  very  violent 
course  in  the  majority  of  cases  and  cases  terminating  fatally  last  only  a 
few  days  or  even  hours.  In  cases  ending  in  recovery  the  disea.se  runs  a 
more  protracted  course,  extending  at  times  over  several  weeks.  Unfav- 
orable cases  terminate  as  the  result  of  spasm  of  the  dia]ihriigiii  or  less 
often  of  exhaustion. 

At  autopsji  nothing  is  found  that  might  be  characteristic  of  death 
from  tetanus.  Even  the  most  careful  examinations  of  the  central  ner- 
vous system  have  up  to  the  present  time  furnished  nothing  positive. 
Whatever  haemorrhages  have  been  found  in  the  spinal  cord,  brain  and 
other  organs,  must  be  attributed  to  stasis  following  obstructed  respi- 
ration. 

The  diagnosis  of  tetanus  is  easily  made  in  both  the  newborn  and 
the  adult.  It  ma}'  be  confused  with  tiie  tonic  spasms  occurring  with 
cerebral  disease.  Such  spasms  occur  in  the  newborn  in  consequence  of 
cerebral  and  meningeal  haemorrhages  and  rarely  with  ])urulent  cere- 
brospinal meningitis,  encephahtis  or  other  cerebral  diseases. 

The  author  recently  saw  a  child  three  weeks  old  with  ciironic  con- 
genital hydrocephalus  in  which  intermittent  tonic  spasms  occurred,  in 
all  the  extremities,  on  moving  or  handling  the  child. 

Congenital  spastic  diplegia  can  give  rise  to  no  confusion  in  diag- 
nosis.- In  doubtful  cases  it  is  advisable  to  make  a  bacteriological  exam- 
ination or  mouse  inoculation.  A  negative  result  proves  nothing  how- 
ever against  the  existence  of  tetanus,  and  then  the  attempt  can  be  made 
to  produce  tetanus  in  the  mouse  by  the  injection  of  the  toxic  lilood. 

The  prognosis  is  in  genera'  verv  unfavoralile.  It  apjiears  to  be 
even  worse  than  with  older  children  and  adults.  Concerning  the  fre- 
quency of  recoveries  reports  vary,  but  on  the  average  probably  20-30 
per  cent,  of  the  cases  recover. 

Prophylaxis  and  Therapy. — Since  we  know  that  tetanus  is  an  in- 
fectious disease,  it  is  our  duty  to  protect  the  navel  wound  by  the  use  of 
aseptic  dressings.  It  also  appears  that  amongst  the  class  of  people  who 
lay  little  stress  on  cleanliness  in  the  care  of  wounds,  tetanus  and  also 
tetanus  of  the  newborn  occur  relatively  with  greater  frequency.  On 
this  fact,  moreover,  probably  depends  the  reported  racial  predisposition 
of  certain  peoples.  When  tetanus  breaks  out  in  a  number  of  persons, 
for  example  in  an  institution  or  a  neighborhood,  it  is  recommended  to 
use  prophylactic  inoculations  with  the  tetanus  antitoxin  discovered  b\- 
Behring  and   Kitasato.     The  tetanus  antitoxin  is  produced   by  injec- 


52  THE    DISEASES    OF    CHILDREN 

tions  of  tetanus  toxin  in  the  horse;  according  to  the  discoveries  of 
Behring  and  Kitasato  the  injection  of  this  toxin  causes  the  appearance, 
in  the  blood-serum,  of  certain  bodies  which  give  the  serum  the  ability 
to  prevent  tetanus,  when  introduced  subcutaneously  into  an  animal 
previously  inoculated  with  tetanus  toxins  or  bacilli.  In  a  few  species 
it  has  also  served  to  cure  the  disease  when  alread}'  active.  The  anti- 
toxin operates  by  combining  with  the  toxin.  The  bacilli  are  not  influ- 
enced, the  antitoxin  not  being  bactericidal.  The  combining  of  tlie 
toxins  and  the  antitoxin  takes  jilace  both  in  vitro  and  in  the  animal 
body.  In  case  however  large  quantities  of  the  toxins  are  already  bound 
to  the  nerve-cells,  the  antitoxin  is  usually  unable  to  break  up  these  com- 
binations and  is  therefore  only  al)le  to  neutrahze  the  poisons  circulating 
in  the  blood.  When  one  remembers  that  even  at  the  onset  of  the  disease 
the  poisons  are  combined  with  the  nerve-cells,  it  becomes  evident  why 
the  serum  therapy  of  tetanus  has  not  given  any  convincing  results  up 
to  the  present  time.  NotwithstantUng  .this,  the  employment  of  the 
serum  must  be  recommended  in  every  case,  inasmuch  as  we  never  know 
how  much,  if  any,  poison  still  remains  in  the  peripheral  nerves  or  is 
possibly  circulating  in  the  blood  or  lymphatic  vessels;  the  poisons  cir- 
culating in  the  blood  can  surely  be  neutralized.  Behring's  antitoxin 
comes  in  bottles  each  containing  250  immunizing  units  (that  is,  such  a 
quantity  of  antitoxin  as  is  capable  of  neutralizing  ten  times  the  mini- 
mal fatal  dose  of  toxin  for  a  guinea-pig).  Besides  this  there  are  other 
rehable  preparations  on  the  market.  The  injection  should  be  given  as 
early  as  possible;  it  is  recommended  to  inject  one  half  the  dose  subcu- 
taneously and  the  other  into  the  brain  (Roux  and  Borrel)  or,  more  sim- 
ply, into  the  subdural  space.  For  this  purpose  the  point  wliich  Quincke 
recommends  for  lumbar  puncture  is  selected,  that  is,  the  space  between 
the  spinous  processes  of  the  second  and  third  or  third  and  fourth  lumbar 
vertebra^.  According  to  Meyer  and  Ransom  no  better  result  can  be 
expected  to  follow  the  subdural  than  the  intravenous  injection  of  the 
antitoxin.  These  authors  consider  injection  of  the  nerve-trunks  to  be 
indicated  in  every  case  in  which  the  point  of  entrance  of  the  infec- 
tion is  known  and  in  which  the  nerves  involved,  as  conductors  of  the 
poison,  are  accessible. 

This  method  of  using  antitoxin  is  not,  however,  available  in  the  new- 
born. The  injection  may  be  repeated,  and  possibly  several  times,  if  no 
beneficial  effects  are  shown  after  twenty-four  hours.  The  serum  has  no 
deleterious  effects;  its  curative  poweis,  according  to  what  has  been 
said,  are  uncertain.  The  injection  of  brain  emulsion,  which,  according 
to  Wassermann,  fixes  the  tetanus  toxin,  has  given  no  beneficial  results; 
therefore  we  must  avail  ourselves  of  those  drugs  which  are  capable  of 
lessening  the  irritabihty  of  the  central  nervous  system.  Of  these  the 
choice  is  chloral.    It  is  given  in  solution,  by  mouth  as  long  as  the  child 


DISEASES    OF    THE    NEWBORN  53 

can  swallow  and  then  by  rectum,  0.5-2.0  Gm.  (8-30  gr.)  per  day.  The 
bromides  and  chloroform  anaesthesia  have  as  a  rule  Uttle  effect.  The  u.se 
of  morphine  in  effective  doses  is  made  difficult  by  reason  of  the  age. 
Monti  recommends  the  subcutaneous  injection  of  the  fluid  extract  of 
physostigma  in  the  dose  of  0.006  Gm.  fyV  gr.)-  Others  recommend  hypo- 
dermics of  atropine,  0.00001-0.00002  Gm.  dnrFO-WTrtr  gr.)  per  day.  Espe- 
cial care  must  be  taken  to  protect  the  cliild  from  being  disturbed  and 
from  cold  inasmuch  as  all  stimuh  further  the  occurrence  of  the  tetanic 
spasms. 

Rest  and  quiet,  enveloping  the  cluld  in  wraps  and  the  use  of  ther- 
mophores are  worthy  of  recommendation.  The  nourishment  requires 
especial  attention.  As  long  as  the  child  can  suck  it  is  put  to  the  breast. 
At  the  very  beginning  of  the  disease,  however,  the  child  sucks  unsatis- 
factoril}^,  or  else  not  at  all.  Then  it  becomes  necessary  to  pump  the 
breast-milk  and  to  feed  the  infant  by  u.sing  a  spoon.  With  artificially 
fed  children  the  same  procedure  is  carried  out  using  modified  cow's 
milk.  Very  soon,  however,  difficulty  arises  in  feeding  the  child  even  b}'' 
the  spoon,  since  the  trismus  interferes.  The  child  nmst  then  be  fed 
through  a  catheter  pa.ssed  through  the  nose  into  the  stomach.  For  this 
purpose  one  employs  a  thin,  soft  catheter,  about  No.  6  Enghsh,  equipped 
with  a  glass  tube  and  with  a  gla.ss  funnel  attached  to  the  latter  by 
means  of  a  soft  rubber  tube.  This  apparatus  must  be  boiled  immediately 
before  each  feeding. 

B.  ERYSIPELAS 

Ery-sipe  as  is  a  wound  infection,  which  leads  to  a  rapidly  spreading 
inflammation  of  the  skin  and  mucous  membranes.  It  arises  at  times 
from  large  wounds  and  at  times  from  insignificant  breaks  or  excoriations 
of  the  skin.  In  the  newborn  the  infection  gains  entrance  as  a  rule 
at  the  umbihcal  wound,  only  very  rarely  elsewhere  and  then  usually 
through  the  skin  of  the  genital  organs.  Since  the  vogue  of  anti-  and 
a-sepsis  in  obstetrical  practice,  the  occurrence  of  erysipelas,  as  of  the 
other  wound  infections  of  the  newborn,  has  become  much  less  frequent. 
Older  cluldren  are  subject  to  the  infection  under  the  same  conditions 
as  adults. 

Symptoms. — In  case  erysipelas  occiu-s  in  a  newborn  infant  the  um- 
bihcal region,  at  the  end  of  the  first  or  beginning  of  the  second  week, 
shows  a  slight  redness  which  spreads  quite  rapidly  downward  over  the 
lower  part  of  the  abdomen.  The  affected  parts  are  very  oedematous  and 
feel  warm;  at  times  pale-red  spots  are  seen  in  their  vicinity.  In  some 
of  the  cases  the  affected  area  has  a  rampart-hke  boundary  which  ad- 
vances i^dth  the  spreading  of  the  disease.  Within  a  few  hours  or  days 
the  disease  advances  over  the  pubic  region  and  the  lower  extremities. 
Only  in  a  small  percentage  of  the  cases  does  erysipelas  of  the  newborn 


54  THE    DISEASES   OF   CHILDREN 

spread  upwards  to  reach  the  chest,  head  and  upper  extremities.  (The 
author  observed  this  only  once  in  ten  cases).  As  a  rule  the  lower 
part  of  the  bod\'  is  the  first  and  most  .severely  affected.  The  disease  is 
not  ahvaj's  accompanied  by  fever.  Chills  are  not  observed  in  the  new- 
born; the  fever  rises  gradually,  in  many  cases  is  very  high,  remains  high 
or  falls  before  death;  in  other  cases  the  disease  runs  its  course  without 
fever.  The  wall-like  boundary  and  the  redness  are  often  not  pronounced 
in  the  newborn.  The  cedema,  however,  is  always  very  marked  and 
tense;  it  may  per.sist  long  after  the  disappearance  of  the  redness  and 
the  healing  of  the  erysipelas;  then  it  is  gradually  absorbed.  Blebs  are 
formed  in  the  skin  just  as  with  erysipelas  in  the  adult.  Not  uncommonly 
the  formation  of  necrotic  areas  in  the  skin  is  seen  in  cases  which  last 
several  days;  these  usually  affect  the  scrotum  or  the  extremities  and 
especially  the  skin  over  the  small  bones  or  on  the  dorsum  of  the  foot. 
In  one  case  observed  by  the  author  there  was  necrcsis  of  the  skin  of  the 
penis  as  well  as  of  the  scrotum.  In  such  cases  circumscribed  areas  of 
the  skin  varying  in  color  from  dark-blue  to  black  occur,  which,  gradu- 
ally becoming  necrotic,  form  rapidly  spreading  ulcers  \nth  a  lardy  sur- 
face.    Phlegmonous  processes  leading  to  suppuration  are  rare. 

Virchow  saw  develop,  in  the  pharynx  of  a  child  with  erysipelas,  an 
acute  phlegmon  which  cau.sed  attacks  of  suffocation. 

Etiology. — The  disease  is  caused  by  the  entrance  of  streptococci 
into  the  skin.  We  owe  the  knowledge  of  this  fact  to  the  investigations- 
of  Fehleisen.  The  streptococcus  of  erysipelas  differs  in  no  way,  how- 
ever, from  the  streptococcus  pyogenes.  Earher  clinical  facts  had  al- 
ready supported  this,  von  Eiselsberg  produced  erysipelas  in  animals  by 
using  streptococci  from  the  pus  of  phlegmons.  Koch  and  Petruschky 
furnished  the  experimental  proof  that  erysipelas  in  man  can  be  pro- 
duced by  using  streptococci  cultivated  from  abscesses.  The  strepto- 
cocci enter  the  lymph-spaces  and  wander  further  in  them.  Infection 
of  the  newborn  probably  takes  place  from  the  hands  of  the  midwife, 
the  nurse,  possibly  also  of  the  physician,  through  the  dressings  and 
clothes.  Children  of  mothers  suffering  from  puerperal  diseases  are 
attacked  ^vith  relatively  gi-eater  frequency.  In  this  connection  the 
assumption  is  justified  that  the  infection  takes  place  from  the  infected 
lochia  of  the  mother,  through  a  third  person  or  through  the  dressings. 

In  one  case  coming  under  the  author's  observation  a  physician  who 
had  dressed  a  patient  with  erysipelas  assisted  at  a  delivery  on  the  same 
day.  The  mother  became  septic  and  the  child  infected  with  erysipelas 
on  the  fifth  day.  In  another  case  a  nursing  baby  was  infected  from 
a  purulent  mastitis  and  developed  an  er3'sipelas  starting  on  the  skin 
of  the  Up. 

Erysipelas  of  the  newborn  is  often  accompanied  by  convulsions 
and  somnolence,  vomiting  and  liquid  stools.     In  some  ca.ses  there  are 


DISEASES   OF   THE   NEWBORN  55 

symptoms  of  sepsis;  inflammatory  processes  of  the  navel  (arteritis, 
etc.)  are  often  demonstrable.  In  occasional  cases  the  abdomen  becomes 
very  tense  and  tender  to  the  touch,  the  oedema  over  it  being  especially 
pronounced  and  highly  distended  veins  being  visible  in  the  skin  of  the 
thorax  and  upper  part  of  the  abdomen.  In  such  ca.ses  one  must  keep 
in  mind  the  possibility  of  an  acute  peritonitis  which  sometimes  (proba- 
bly as  a  result  of  the  general  .septic  infection)  accompanies  erysipelas 
neonatorum.  This  complication  I  have  observed  in  two  cases.  Erysip- 
elas in  older  children  varies  in  no  way  from  that  in  the  adult.  High 
fever  accompanying  it  is  the  rule.  Glandular  swellings  are  usually 
demonstrable.  It  remains  only  to  call  attention  to  the  now  rare  occur- 
rence of  vaccination  erysipelas.  Formerly,  in  the  days  of  humanized 
virus,  this  appeared  as  veritable  epidemics. 

Course  and  Progress. — Erysipelas  neonatorum  spreads  very  rapidly, 
as  a  rule,  and  with  few  exceptions  results  in  death  with  manifestations 
of  cardiac  weakness.  In  older  children  the  course  is  usually  less  violent 
and  recovery  takes  place  much  oftener.  With  them  also  we  not  uncora^ 
monly  encounter  relapses  and  recurrences. 

Pathology. — In  the  diseased  areas  besides  the  hyperannia,  an 
extensive  oedema  of  all  the  layers  of  the  skin  is  found  with  small  round- 
celled  infiltration  of  the  skin  and  sulicutaneous  tissues.  Also  lymph- 
node  swellings,  enlarged  spleen  and  parenchymatous  degeneration  of 
the  glandular  organs.  In  many  cases  in  the  newlaorn  diseases  of  the 
umbilical  vessels  and  sometimes  sepsis  can  be  anatomically  demon- 
strated. In  uncomplicated  cases  innumerable  foci  of  streptococci  outside 
of  the  blood  vessels  in  the  affected  area  may  be  detected. 

Therapy. — The  treatment  of  erysipelas  in  general,  promises  no 
brilliant  results.  The  treatment  with  antistreptococcus  serum  as  inaug- 
urated by  Marniorek.  though  theoretically  well  grounded,  has  completely 
failed  in  practice.  Attempts  to  prevent  the  spreading  of  the  erysipelas, 
by  sealing  the  affected  area  and  vicinity,  have  had,  in  the  main,  no 
definite  results.  For  this  purpose  oil-paints,  varnish,  shellac,  (siccative, 
Gersuny)  and  gutta-percha  tissue  have  been  recommended.  Wolfler 
applies  strips  of  adhesive  plaster  at  a  short  distance  from  the  boundary 
of  the  affected  area,  for  the  purpose  of  compressing  the  13'niph-spaces 
and  thus  furnishing  a  hindrance  for  the  migration  of  the  streptococci. 
As  a  rule  moist  dressings  are  employed  and  compresses  of  liquor  alum- 
inii  acet.  (P.  G.)  diluted  8  times,  or  alcohol  50  per  cent.,  sublimate 
1-1000,  boracic  acid  1-2  per  cent.,  salicylic  acid  1  per  cent.,  or  lead- 
water.  The  use  of  cold  applications  is  often  satisfactory  as  they 
usually  at  least  reheve  the  pain.  Others  apply  ichthyol  ointment 
and  recommend  painting  with  tincture  of  iodine  (a  procedure  to  be 
avoided  in  the  infant).  The  artificial  hyperemia  of  Bier  seems  to 
promise  results. 


56  THE   DISEASES   OF   CHILDREN 

C.  OPHTHALMIA  NEONATORUM 
As  ill  older  children,  blennorrhcea  of  the  newborn  is  an  acute  infec- 
tious inflammation  of  the  conjunctiva  which  is  characterized  by  profuse 
purulent  secretion  and  granular  infiltration.  The  method  of  infection 
in  the  newborn,  however,  is  unique  and  the  disease  must  be  classed 
amongst  the  infections  due  to  parturition. 

Symptoms. — In  the  great  majority  of  cases  the  disease  begins  on 
the  second  or  third  day  of  life,  with  cedema  and  slight  retlness  of  the 
lids,  usually  of  both  eyes,  however,  in  one  fourth  to  one  fifth  of  the 
cases  only  in  one  eye.  The  oedema  gradually  increases  until  the  skin  of 
the  lids  becomes  extremely  tense;  the  hds  are  no  longer  opened  spon- 
taneously and   are  separated  with  difficulty,  by  the  physician. 

At  this  time  a  fairly  profuse,  thin  secretion  commences,  resembling 
in  color  washings  from  meat,  and  containing  a  few  floccuh.  Inspection 
of  the  conjunctiva  shows  severe  hypera^mia  and  swelling,  not  confined 
to  the  palpebral  portion  and  the  fornix  but  extending  also  to  the  globe; 
here  the  process  is  usually  least  marked,  but  it  occasionally  leads  to 
extensive  swelling,  limited  sharply  at  the  corneal  margin  (chemosis). 
Small  ha?morrhages  in  the  conjunctiva  are  not  uncommon.  Two  or 
three  days  after  the  beginning  of  the  disease  the  secretion  becomes 
thicker,  lemon-  to  greenish-yellow,  creamy  and  very  profuse.  The 
oedema  of  the  hds  gradually  diminishes,  the  redness  subsides,  and  the 
surface  of  the  conjunctiva,  smooth  up  to  this  time,  becomes  granular; 
the  fornix  becomes  thickened  and  plump  and  bulges  forward  on  evert- 
ing the  lids.  After  two  to  four  weeks  the  suppuration  diminishes,  the 
granulations  on  the  conjunctiva  subside  and  its  surface  becomes  smooth 
again;  the  secretion,  losing  its  strictly  purulent  character,  becomes  first 
muco-purulent  and  less  profuse  and  finally  catarrhal;  the  hypersemia 
lessens  and  the  conjunctiva  returns  to  its  normal  state;  scar  formation 
does  not  usually  occur,  even  after  the  severest  processes. 

In  many  cases  the  inflammation  does  not  extend  so  far;  the  purulent 
secretion  is  scant  and  may  even  be  entirely  wanting;  in  other  cases  the 
manifestations  are  severer,  the  oedema  especially  exten.sive,  the  inflamma- 
tion leading  to  the  formation  of  pseudomembranes  on  the  tarsal  conjunc- 
tiva and  occasionally  to  the  occurrence  of  membranous  tissue  necrosis. 

The  severest  complication  is  the  involvement  of  the  cornea  in  the 
inflammatory  process:  this  has  now,  with  the  timely  inauguration  of 
the  proper  therapy,  happily  become  a  rarity.  Infiltrations  are  formed 
at  times  on  the  margin  and  at  times  in  the  centre  of  the  cornea;  the 
superficial  epithelium  of  the  cornea  becomes  dull  and  suppurative  solu- 
tion of  the  tissues  takes  place  with  the  formation  of  an  ulcer  which  usu- 
ally perforates.  The  central  ulcers  do  not  usually  spread  after  perfora- 
tion, but  in  some  cases  the  entire  cornea  is  involved.  Marginal  ulcers 
are  easily  overlooked  when  they  are  covered  by  the  swollen  conjunc- 


DISEASES    OF   THE    NEWBORN  57 

tiva;  they  are  either  single  or  when  multiple  can  become  confluent, 
causing  the  loss  of  the  entire  cornea.  The  consequences  of  perforation 
differ  according  to  its  location;  total  or  partial  staphyloma,  panophthal- 
mitis with  destruction  of  the  bulb,  capsular  cataract,  anterior  synechia^. 
The  corneal  involvement  usually  occurs  between  the  5th  and  14th  daj- 
of  the  disease.  As  a  rule  the  earlier  the  cornea  becomes  involved  the 
severer  is  the  disease. 

Ophthalmia  neonatorum  is  often  accompanied  by  disturbances  of 
the  nutrition  of  the  child  and  by  fever.  Except  for  the  extension  to  the 
cornea,  complications  are  rare.  One  of  the  most  important  is  arthritis, 
caused  by  the  entrance  of  the  gonococcus  into  the  circulation.  The 
specific  arthritis  has  as  its  seat  of  predilection  the  knee-joint;  it  may, 
however,  attack  other  joints,  such  as  the  wrist,  hip,  ankle  and  shoulder. 
Sometimes  mono-  and  sometimes  polyarticular,  it  leads  to  painful  swell- 
ing in  the  neighborhood  of  the  joints  which  become  hot  and  oedem- 
atous.  Active  movements  are  either  not  carried  out  at  all  or  else  only 
within  a  limited  range;  passive  movements  are  resisted  and  cause  great 
pain.  There  is  usually  a  serous  effusion  into  the  joint  cavity.  In  some 
cases  suppuration  occurs  in  or  around  the  joint.  The  arthritis  runs  a 
febrile  course  in  some  cases;  in  the  majority,  however  it  is  afebrile. 
Permanent  impairment  to  joint  functions  does  not  occur.  Mgnaudon 
reports  muscular  atrophies  occurring  as  the  result  of  this  arthritis. 

The  causative  agent  of  the  ocular  inflammation,  the  gonococcus, 
has  been  demonstrated  in  the  joint  fluid  in  a  number  of  cases,  first  by 
Deutschmann;  in  two  cases  (Sobotka,  Finger)  streptococci  were  found 
with  the  gonococcus. 

Gonococcus  stomatitis  is  the  rarest  complication  of  ophthalmia 
neonatorum;  it  may  occur  from  the  infected  tears  running  off  through 
the  lachrymal  ducts,  or  possibly  from  contamination  of  the  oral  cavity 
directly.  It  is  manifested  b)'  extensive  exudation  and  purulent  secre- 
tion; gonococci  have  been  found  in  the  secretion  (Rosinski). 

Etiology. — In  the  vast  majority  of  cases  conjunctival  blennorrhoea 
of  the  newborn  is  produced  by  the  gonococcus  of  Neisser.  In  44  per 
cent,  of  observed  ca.ses  Groenow  demonstrated  the  gonococcus;  Amnion 
in  56  per  cent,  and  Haupt  in  71  per  cent.  When  we  consider  the  fact 
that  in  cases  of  longer  duration  we  are  not  able  to  find  the  gonococcus, 
the  percentage  of  cases  caused  by  the  gonococcus  must  be  even  higher 
than  the  above.  The  remaining  cases  of  purulent  conjunctivitis  in  the 
newborn  are  caused  by  the  bacteria  of  inflammation;  pneumococcus, 
streptococcus,  bacillus  coli,  bacillus  pseudo-influenztr  and  possibly 
staphylococcus  pyogenes  aureus  and  others.  Saemisch  advises  cata- 
loging the  cases  of  purulent  inflammations  of  the  newborn  not  due  to 
the  gonococcus  as  acute  blennorrhceic  conjunctivitis,  in  contradistinction 
to  acute  gonoblennorrhoea. 


58  THE    DISEASES   OF   CHILDREN 

Pathogenesis. — Infection  of  the  conjunctiva  of  the  newborn  can 
take  pUice  in  various  ways.  As  a  rule  the  infection  takes  place  from  a 
ui'ethritis  or  vaginitis  of  the  mother.  Herewith  the  child  may  be  already 
infected  in  utero  or,  through  the  agency  of  the  examining  physician  or 
midwife  whose  fingers  carry  the  infection  from  the  vagina  to  the  eyelids 
of  the  child,  and  possibly  also  through  infected  anmiotic  fluid.  Usually, 
however,  the  child  is  infected  during  the  passage  of  the  head  through 
the  birth  canal,  when  the  lids  become  covered  with  the  secretion  wliich 
reaches  the  conjunctiva  when  the  eyes  are  opened. 

In  many  cases  the  child  is  infected  post  partum  from  the  hands  of 
the  nurse  or  mother.  In  institutions  children  of  healthy  mothers  are 
sometimes  infected  indirectly  from  other  chiUlrcn  infected  with  blen- 
norrha^a. 

Experience  teaches  that  the  cases  of  ophthalmia  arising  from  infec- 
tion during  birth  only  rarely  begin  later  than  the  fifth  day. 

Diagnosis. — The  diagnosis  of  gonorrhoeal  ophthalmia  is  easily  estab- 
hshed.  It  can  easily  be  differentiated  from  simple  catarrh  caused  by 
irritation  (nitrate  of  silver  catarrh)  of  the  conjunctiva  and  from  puru- 
lent conjunctivitis  caused  by  other  than  gonorrhceal  infection,  by  means 
of  bacteriological  examinations.  In  some  cases  an  acute  dacryocystitis, 
such  as  occasionally  occurs  in  the  newborn  as  the  result  of  a  congenital 
closure  of  the  lachrymal  duct,  may  give  rise  to  confu.sion  with  gono- 
blennorrhoea.  With  this  condition,  however,  the  disease  is  always  uni- 
lateral and  by  pressure  over  the  tear  sac  pus  may  be  expressed. 

Frequency. — Ophthalmia  neonatorum  is  very  prevalent,  but  owing 
to  the  prophylactic  measures  employed  in  maternities  it  has  become  less 
frequent.  Notwithstanding  this,  however,  according  to  the  statistics  of 
Cohn  for  the  year  1906,  there  are  stall  31  per  cent,  of  the  inhabitants  of 
asylums  for  the  l^lind  in  Germany,  who  owe  their  lo.ss  of  vision  to  gono- 
blennorrhcea. 

The  prognosis  is  good,  when  timely,  suitable  treatment  is  instituted. 
The  non-gonorrhoeal  purulent  inflammations  lead  to  involvement  of  the 
cornea  less  often   than  the  gonorrhoeal. 

Prophylaxis  is  most  important.  Excellent  results  have  been  at- 
tained from  the  procedure  introduced  by  Crede.  According  to  his  ad-' 
vice,  the  infant  should  be  cleansed  and  bathed  immediately  after  birth; 
the  Hds  cleansed  externally  with  sterile  water  applied  with  cotton  pled- 
gets; then  one  drop  of  a  2  per  cent,  silver  nitrate  solution  should  be 
applied  to  each  eye  by  means  of  a  glass  rod:  this  appHcation  should 
not  be  repeated. 

Crede's  method  has  given  admirable  results  and  has  unquestionably 
reduced  the  frequency  of  blennorrhoea  neonatorum.  Since,  however,  its 
employment  is  not  absolutely  preventive,  it  is  recommended  to  use  re- 
peated disinfecting  vaginal  douches  during  delivery.     Many  clinicians 


DISEASES    OF    THE    NEWBORN  59 

lay  especial  value  on  the  cleansing  of  the  lids  externally  with  disinfect- 
ing fluids  immediately  after  birth,  and  some  even  prefer  it  to  Crede's 
method.  Objection  to  the  latter  method  is  often  raised  on  the  ground 
that  it  frequently  leads  to  irritation  of  the  mucosa  (silver  nitrate  ca- 
tarrh). On  these  grounds  a  1  per  cent,  solution  of  silver  nitrate  is  often 
used.  Substitutes  for  the  nitric  acid  salt  of  silver  .seem  only  to  be  le.ss 
effective  than  the  latter  itself. 

Crede's  method  has  doubtle.ss  been  of  great  value  in  maternity  ho.s- 
pitals.  The  question  as  to  whether  it  should  he  generally  employed  in. 
private  practice  and  possibly  made  obligatory,  is  still  under  lively  dis- 
cussion. It  should  be  used,  however,  in  conjunction  witli  vaginal 
douches,  in  every  case  in  which  the  physician  or  midwife  detects  a  puru- 
lent vaginal  discharge  in  the  niotlier  before  delivery. 

Late  infections  can  be  absolutely  avoided  by  scrupulous  cleanliness 
on  the  part  of  nurse  and  attendants.  Nurses  and  mothers  should  be 
admonished  to  cleanse  their  hands  thoroughly  every  time  before  hand- 
ling the  child.  It  is  self-evident  that  mother  and  child  should  not  em- 
ploy in  common  such  things  as  washing  utensils,  syringes,  cotton,  etc. 

Therapy. — The  treatment  of  gonoblennorrha>a  should  be  directed 
toward  lessening  the  inflammation  and  removing  as  quickly  as  possible 
the  accumulating  .secretion.  To  prevent  corneal  comphcations  ice  com- 
presses are  used  in  the  first  stages  of  the  disease  and  kept  up  until  the 
secretion  becomes  .shght  and  its  purulent  character  has  disappeared. 
For  this  purpose  small  gauze  or  hnt  compresses  folded  four  to  eight 
times  are  used.  Many  of  these  are  prepared,  put  on  ice  and  changed 
every  three  to  five  minutes.  Since  the  gauze  or  hnt  absorbs  the  .secre- 
tion, the  compresses  must  be  frequently  replaced  by  new  ones.  When 
changing  the  compresses  the  secretion  should  be  wiped  away  with  sterile 
cotton.  The  secretion  collecting  in  the  fornix  should  be  removed  by 
douching.  The  douches  should  be  used  very  often  when  suppuration 
has  begun,  every  one-half  to  one  hour,  day  and  night.  It  is  in  this 
connection  of  great  importance  to  avoid  any,  even  the  slightest,  injury 
to  the  corneal  epithelium,  in  order  to  avoid  pa^•ing  the  way  for  infiltra- 
tions of  the  cornea. 

The  following  solutions  are  recommended  for  douching:  potassium 
permanganate  in  a  red  (3  per  cent.),  or  in  very  dilute  solution;  subli- 
mate 1:5000;  boracic  acid  three  per  cent;  oxycyanate  of  mercury 
1:  2000;  also  physiological  sahne  solution;  boiled  water  and  others. 

With  the  beginning  of  the  secretion,  the  conjunctiva  should  be 
swabbed  daily  with  a  2  per  cent,  solution  of  silver  nitrate.  Toucliing 
the  cornea  with  the  silver  solution  and  the  u.sc  of  sahne  to  neutralize  it, 
should  be  carefully  avoided.  In  case  the  inflammatory  signs  and  the 
secretion  should  not  soon  diminish,  one  can  employ  a  3  per  cent,  solu- 
tion of  silver  nitrate  for  application  to  the  conjunctiva.    If  only  one  eye 


60  THE   DISEASES   OF   CHILDREN 

is  attacked  the  healthy  eye  can  be  protected  against  infection  by  drop- 
ping into  it  daily  one  drop  of  a  1  per  cent,  silver  nitrate  solution,  lian- 
daging  the  healthy  eye  is  very  efficient,  but  irritations  of  the  skin 
and  also  of  the  conjunctiva  occur  quite  readily  under  the  bandage. 
Notwithstanding  this,  bandaging  the  healthy  eye  is  often  advocated 
when  only  one  eye  is  affected. 

When  the  cornea  is  involved,  1  per  cent,  atropine  solution  (when  the 
ulcer  is  marginal,  1  per  cent,  eserin)  should  be  dropped  into  the  eye. 
moreover  the  use  of  the  ice  compresses  must  be  discontinued;  but  not 
the  application  of  silver  nitrate.  One  should  consult  the  text  books  on 
ophthalmology  for  further  information  on  this  topic. 

Arthritides  are  treated  by  application  of  liquor  aluminii  acetici 
(P.  G.)  diluted  eight  times.  It  is  best  first  to  anoint  the  skin,  over  the 
diseased  joints,   with  vaseline. 

Purulent  effusions  into  the  joints,  manifested  by  extensive  inflam- 
matory signs  and  established  by  exploratory  puncture,  are  to  be  incised 
under  aseptic  precautions.  Salicylates  may  be  tried  but  seem  to  accom- 
plish very  little. 

D.     SEPSIS  IN  INFANCY 

The  lack  of  unanimity  concerning  the  septic  diseases,  renders  it 
advisable  at  the  outset  to  fix  distinctly  just  what  is  understood  by  the 
term  "sepsis." 

From  a  great  number  of  definitions  which  up  to  the  present  have 
not  fully  succeeded  in  classifying  the  subject,  we  prefer  that  of  Len- 
hartz,  and  will,  therefore,  understand  with  him  "under  the  caption 
sepsis,  all  general  diseases  caused  by  the  pyogenic  cocci  and  other  equiv- 
alent bacteria."  Whereas,  rather  inappropriately,  the  septic  processes 
without  abscess  formation  are  to  be  termed  sepsis,  the  ancillary  term 
"with  metastases"  is  to  be  employed  when  reference  is  made  to  what 
has  heretofore  been  known  as  pya-mia  (metastasizing  sepsis).  AVe. 
however,  only  speak  of  metastasizing  sepsis  when  proof  is  present  either 
during  life  or  at  necropsy  that  the  abscess  has  not  been  formed  by  di- 
rect contiguity  through  the  lymph-  and  tissue-spaces,  but  has  occurred 
through  the  medium  of  the  circulation.  Kocher  and  Tavel  speak  of 
bacteriiEmia  when  bacteria  have  gained  entrance  and  circulate  in  the 
vascular  system,  and  of  toxa-mia  when  only  products  of  bacterial 
metabolism  have  been  absorbed  into  the  circulation.  On  account  how- 
ever of  the  varying  symptoms  of  sepsis  in  the  first  months  of  fife,  often 
difficult  of  interpretation,  we  must  limit  the  designation  sepsis  without 
exception  to  those  disease  pictures  in  which  proof  has  been  furnished 
of  the  presence  of  bacteria  in  the  blood.  When  in  a  given  case  no  proof 
is  adduced  or  can  be  furnished  of  the  presence  of  bacteria  in  the  circula- 
tion, we  are  only  justified  in  classifying  it  under  the  head  of  sepsis  when 


DISEASES    OF    THE    NEWBORN  61 

it  agrees  in  symptom-complex,  course,  and  possibly  in  anatomical  find- 
ings, with  those  disease  pictures  in  which  general  bacterial  infection  has 
been  demonstrated. 

The  question  to  what  extent  we  are  justified  in  attributing  the 
symptom-complex  of  sepsis  to  a  mere  toxaemia  must  be  held  in  abeyance. 
It  is  to  be  expected  that  improvement  in  our  bacteriological  technique 
will  enable  us  to  reserve  the  designation  sepsis  for  that  symptom-com- 
plex with  which  the  entrance  of  pyogenic  organisms  into  the  circulation 
has  been  demonstrated.  This  proof  can  already  be  furnished  in  most 
cases,  if  not  during  life  at  least  at  necropsy. 

Cause  of  Sepsis. — Sepsis  in  the  infant,  as  in  the  adult,  may  be  caused 
by  a  variety  of  bacteria.  First  and  foremost  the  so-called  pyogenic 
cocci  are  to  be  named.  The  staphylococcus  pyogenes  albus  and  aureus, 
the  streptococci  (among  which  the  intestinal  streptococcus  of  Esclierich 
occupies  a  prominent  place),  the  diplococcus  pneumoniae  (Fraenkel- 
Weichselbaum),  bacillus  coli  communis,  bacterium  lactis  aerogenes, 
bacillus  ehteritidis  Gartner  and  related  stems,  bacillus  pyocyaneus,  the 
proteus  group,  more  rarely  the  gonococcus,  the  influenza  bacillus  of 
Pfeiffer  and  possibly  the  bacillus  of  Friedlander  and  the  meningococcus 
of  Weichselbaum. 

Varieties  of  Sepsis. — Sepsis  may  be  primary,  or  secondary  to  a  pre- 
existing disease.  It  is  usually  caused  by  a  single  organism  and  only 
occasionally,  although  seemingly  more  often  in  the  infant  than  the 
adult,  there  is  a  polymicrobic  infection. 

We  must  differentiate  a  hetero-  and  auto-infection  just  as  in  the 
adult.  According  to  Kocher  and  Tavel  we  speak  of  hetero-infection 
when  the  cause  of  the  disease  comes  from  without  and  of  auto-infec- 
tion when  it  is  already  present  in  the  organism  under  normal  conditions, 
before  the  disease.  The  majority  of  infections  are  hetero-infections. 
The  infant  with  its  special  susceptibihty  has  ample  opportunity  for 
contamination  with  infectious  agents;  especially  those  from  the  air; 
also  through  its  clothes  and  further  through  its  food,  which  in  infancy 
is  usually  milk.  Mothers'  milk  is  nuich  better  protected  against  infec- 
tion than  cow's  milk.  We  know,  however,  that  the  milk  of  healthy  as 
well  as  septic  mothers  contains  bacteria.  The  bacteria  enter  the  lacteal 
ducts  from  without;  according  to  Basch  and  Weleminsky  bacteria  never 
invade  the  milk  from  the  circulation  unless  there  has  been  some  distur- 
bance of  the  structure  of  the  breast  (e.g.,  ha-morrhage).  Pyogenic  bacteria 
are  regularly  found  in  the  milk  of  animals.  The  infant  is  also  exposed 
to  infection  from  the  water  used  in  cleansing  its  mouth  and  perhaps 
even  from  the  water  of  the  bath;  this  latter  source  of  infection  has 
hardly  been  estabUshed  and  has  been  at  all  events  much  over-estimated 
in  importance.  The  newborn,  moreover,  is  exposed  to  infection  from 
the  lochia,  which  normally  contains  pathogenic  bacteria;  this  infection 


62  THE    DISEASES   OF   CHILDREN 

either  takes  place  directly  during  the  passage  of  the  child  through  the 
birth  canal,  or  indirectly,  through  carrying  the  lochia  to  the  body  of 
the  child  by  mother  or  nurse  or  on  instruments  or  bandages. 

The  newborn,  moreover,  as  also  the  older  child,  is  exposed  to  infec- 
tion from  other  children,  especially  infants  suffering  from  septic  or 
inflammatory  disease;  the  infection  being  carried  by  ph3'sicians,  atten- 
dants and  on  utensils.  The  frequent  occurrence  of  veritable  epidemics 
of  sepsis  of  the  newborn  in  maternities  can  be  easily  understood. 

Susceptibility. — Infants  possess  seenringly  a  relatively  low  resis- 
tance to  septic  infection;  the  younger  the  child  the  less  the  resistance 
and  the  less  mature  the  child  at  birth  the  less  its  resistance.  Thus  the 
premature  infant  is  especially  menaced  by  septic  infection.  It  is 
also  a  very  striking  fact  that  infants  seem  to  become  septic  less  often 
when  at  home  than  in  institutions.  This  reminds  us  of  the  behavior  of 
puerperal  fever;  yet,  whereas,  puerperal  fever  belongs  to  the  rarities  in 
well  conducted  maternities,  sepsis  neonatorum  is  still  not  an  uncommon 
occurrence  and  epidemics  of  this  disease  are  a  menace  to  every  hospital 
for  the  care  of  infants.  The  low  protective  power  which  the  nurshng 
develops  against  septic  disease  is  partly  attributed  to  the  undeveloped 
condition  of  its  organs.  Thus  the  lymph-nodes,  which  play  a  prominent 
part  in  the  defence  against  sepsis,  arc  of  little  or  no  importance  to  the 
nursling  and  the  absence  of  even  regional  lymph-node  enlargement  is 
the  rule  in  sepsis  neonatorum.  Further,  we  must  consider  the  unde- 
veloped condition  of  the  skin,  which  seems  especially  adapted  for  pro- 
tection against  infection  (according  to  Hulot  the  stratum  corneum  is 
scantily  developed  in  the  newborn).  The  epithehum  of  the  gastro- 
intestinal tract  is  also  said  to  be,  in  contradistinction  to  the  adult,  per- 
vious for  bacteria  even  without  any  lesion.  For  certain  animals  it  has 
been  firmly  established  that  the  intestinal  mucosa  of  the  newborn  is 
not  impervious  to  the  passage  of  certain  bacteria;  whereas  this  is  not 
the  case  in  the  adult  animal;  to  wiiat  extent  this  holds  good  in  human 
beings  has  not  been  established.  The  middle  ear  is  also  not  fully  devel- 
oped, the  tympanic  cavities  being  filled  with  an  endoryonal  tissue  re- 
sembling the  jell}'  of  Wharton  in  its  structure.  We  can  attribute  the 
susceptibility  of  the  young  infant  .to  septic  infection  principally,  perhaps, 
to  its  insufficiently  or  scantily  developed  capacity  for  manufacturing 
protective  substances  (Halban,  Landsteiner). 

The  well-established  observation  that  artificially  fed  infants  often 
succumb  to  septic  infection,  whereas  breast-fed  children  are  relatively 
seldom  attacked  (which  holds  good  for  the  nurshng  after  the  end  of  the 
second  month)  must  have  direct  relation  to  the  food.  Explanatory  of 
this,  we  can  refer  to  Moro's  investigations  concerning  the  transmission 
of  alexins  from  the  mother's  milk,  rich  in  these  substances;  as  cow's  milk 
is  much  poorer  in  alexins  we  could  thus  explain  the  increased  protection  of 


DISEASES    OF    THE    NEWBORN  63 

breast-fed  children.  The  beHcf  is  also  advanced  that  the  cells  of  the 
artificially  nourished  child  are  so  taxed  by  assimilation  of  the  foreign  or 
aspecific  ("artfrenid")  food  that  they  can  elaborate  less  protective  sub- 
stance against  possilile  infection. 

In  the  nursling,  during  the  first  days  of  life,  the  umbilical  cord  and 
the  wound  left  after  its  separation  are  especially  liable  to  infection. 
Infection  of  the  newborn  is  further  facilitateil  by  the  physiological  des- 
quamation of  the  skin  and  mucous  membranes,  .\fter  consideration  of 
all  these  conditions  it  seems  clear  that  the  newborn,  particularly  the 
premature  newborn  is  especially  susceptible  to  general  septic  infection. 

The  susceptibihty  for  sepsis  is  doubtless  increased  by  the  presence 
of  other  diseases,  among  which  hereditary  syphilis  and  intestinal  affec- 
tions are  most  important  in  infants. 

Portals  of  Entry. — The  newl)orn  infant  may  Ix'  born  septic.  The 
virus  can  pass  to  the  foetal  circulation  through  the  placenta,  when  bac- 
teria, which  have  broken  through  the  placental  vessels,  circulate  in  the 
mother's  blood.  Such  cases  have  been  verified  not  only  for  the  pyogenic 
cocci  but  also  for  other  bacteria,  e.g.,  bacillus  typhosus,  diplococcus 
pneumoniae,  etc.  The  newborn  can  also  become  septic  through  aspira- 
tion of  infected  liquor  amnii,  as  when  the  bag  of  waters  ruptures  too 
earl}'.  The  newborn  can  further  be  infected  during  its  passage  through 
the  birth  canal. 

Dubrisay  reported  a  case  of  purulent  vaginitis  in  the  mother;  sepsis 
of  the  child;  death  11  hours  after  birth  with  pleurisy  and  pneumonia. 
Congenital  and  placental  infections  and  sepsis  acquired  during  the  birth 
are  frequent  experiences. 

After  birth,  the  luiihiUcal  cord  is  the  most  frequent  point  of  entry 
for  sepsis.  Infection,  moreover,  takes  place  usually  before  the  separa- 
tion of  the  cord.  After  separation  of  the  cord  the  navel  wound,  for  rea- 
sons given  in  the  chapter  on  diseases  of  the  navel,  often  furnishes  the 
gateway  for  local  and  general  infection.  Next  in  frequency  as  an  en- 
trance port  for  infection  comes  the  skin,  its  physiological  ]ieculiarities 
in  the  newborn,  the  many  traumata  to  which  it  is  exposed  and  which 
so  often  lead  to  superficial  diseases  (eczema,  fiuainculosis)  predisposing  it 
to  septic  infection. 

Fissures,  sUght  tears,  superficial  abrasions  of  the  epithelium,  such 
as  are  caused  by  mechanical  cleansing,  decubitus  on  the  heel  or  above 
the  internal  malleoli  or  over  the  sacrum,  such  as  often  develop  in  chil- 
dren suffering  from  disturbances  of  nutrition,  are  convenient  gateways 
for  the  entrance  of  the  virus.  The  mucosa  of  the  oral  cavity  is  very 
often  the  starting  point  of  sepsis  (Epstein).  The  phy.siological  shed- 
ding of  the  mucosa  in  the  newborn,  mechanical  abrasions  due  to  clean.s- 
ing  of  the  mouth,  fissures  of  the  mucosa,  the  so-called  Hednar's  aphthiie, 
septic  pseudodiphtheritic  inflammation  of  the  oial   mucosa   (Epstein), 


64  THE    DISEASES   OF    CHILDREN 

thrush,  and  the  various  forms  of  stomatitis  and  gingivitis  can  all  lead  to 
sepsis.  The  pharyngeal  and  nasal  mucous  membranes,  as  a  rule  only 
when  locally  diseased,  are  also  at  times  the  starting  point  of  general 
infection.  It  is  worthy  of  note  that  the  tonsils  have  only  in  the  rarest 
instances  been  considered  the  entrance  for  general  septic  infection.  In 
very  rare  instances  sepsis  arises  from  the  conjunctiva  and  that  too,  only 
when  it  is  the  site  of  some  inflammation,  gonococcus  or  other. 

The  ear,  which  is  a  frequent  site  of  local  disease  in  the  infant,  can 
also  be  the  source  of  a  sepsis  prone  to  have  the  clinical  picture  of  puru- 
lent meningitis   (Scherer). 

The  mucosa  of  the  gastro-intestinal  tract  has  been  regarded  by  many 
as  the  starting  point  of  septic  processes.  Sevestre  and  others  have  con- 
sidered the  inflamed  intestine  to  be  the  point  of  entry  and  Czerny  and 
Moser  have  held  gastro-enteritis  to  be  the  primary  focus  of  a  general 
sepsis.  Proof  however,  has  not  yet  been  adduced  and  only  the  few  well 
established  cases  in  which  the  intestinal  streptococci  of  Escherich  have 
lead  to  sepsis  serve  as  a  foundation  for  the  opinion  that  the  newborn 
and  young  child  can  be  infected  with  sepsis  starting  from  the  injured 
intestinal  mucosa. 

Very  much  more  importance  must  be  attributed  to  the  lungs  than 
to  the  gastro-intestinal  tract,  as  furnishing  the  primary  focus  of  sepsis 
(Fischl). 

It  is  usually  either  a  bronchitis  with  necrosis  of  the  epithelium  or 
else  more  or  less  extensive  inflammatory  foci,  from  which  the  infection 
spreads  by  way  of  the  lymphatics  (Fischl). 

In  some  cases  the  infection  can  be  referred  to  injury  at  birth;  namely, 
when  the  injury  has  led  to  local  inflammation.  In  other  cases  the  skin 
is  the  gateway  for  the  entrance  of  the  infection;  and  diseases  of  the 
skin  often  furnish  the  chance  for  septic  infection. 

In  relatively  infrecjuent  instances  local  infections  of  the  vulva, 
more  frequently  diseases  of  the  bladder,  and  cystitis,  by  extension  to 
the  upper  urinary  tract,  can  give  rise  to  septic  infection  (Escherich, 
Trumpp).  It  is  worthy  of  note  that  this  mode  of  infection  (cystitis)  is 
almost  without  exception  confined  to  female  infants. 

In  many  cases  the  starting  point  of  the  sepsis  remains  unknown: 
we  may,  therefore,  speak  of  a  cryptogenic  sepsis. 

Clinical  Picture. — Sepsis  occurs  in  various  forms  in  the  infant, 
dependent  on  the  origin  of  the  infection,  the  age  of  the  child  and  the 
virulence  of  the  bacteria. 

We  must  first  note  that  there  are  many  cases  which  run  their  course 
practically  without  symptoms.  With  or  without  a  demonstrable  primary 
disease,  while  apparently  well,  the  child  goes  into  sudden  collapse  and 
dies,  the  temperature  falhng  rapidly.  In  such  cases  the  diagnosis  of  a 
general  infection  can  only  be  suspected,  though  somewhat  more  strongly 


DISEASES  OF  THE  NEWBORN  65 

when  the  newborn  is  premature.  In  other  cases,  however,  the  course 
of  the  disease  is  stormy,  with  a  higii  fever,  vomiting  and  severe  diar- 
rhoea, so  that  the  sepsis  runs  its  course  under  the  clinical  picture  of  an 
acute  gastro-enleritU  (v.  Ritter,  Epstein).  In  a  third  group  of  infants, 
we  have  fever,  ashen  gray  pallor  of  the  skin,  haemorrhages  in  the  skin 
and  organs,  severe  inflammatory  signs  at  the  navel,  on  the  skin,  in  the 
lungs,  gastro-intestinal  or  urinary  tract.  Added  to  these  there  are  se- 
vere disturbances  of  the  central  nervous  system  which  can  also  quite 
dominate  the  pictiu'e,  so  that  the  disease  can  sinuilate  severe  intoxica- 
tion or  meningenJ  di>ieafte.  In  other  cases  purulent  metastases  form  the 
salient  features  of  the  disease  or  else — and  especially  in  asylums — pul- 
monary symptoms  are  so  prominent  that  the  disease  runs  a  course  under 
the  clinical  picture  of  a  pneumonia.  Of  special  importance  are  the  cases 
in  which  hajmorrhages  into  the  skin  or  internal  organs  form  the  most 
salient  and  often  the  only  symptom  of  septic  disease.  A  number  of 
cases  which  run  their  course  under  the  picture  of  mehrna  must  be  classed 
with  sepsis.  Other  cases  are  classified  under  the  heading  of  "unibihcal 
haemorrhages"  because  the  bleeding  from  the  navel  is  the  dominating 
and  perhaps  the  only  symptom  and  because  no  proof  of  sepsis  is  sought 
for  or  furnished  either  during  life  or  at  necropsy. 

The  probal)ility  of  sep.sis  increases  when  the  hiemorrhage  is  not 
single  or  from  only  one  organ,  but  multiple.  This  leads  us  to  the  symp- 
tom-complex which  has  taken  a  place  in  the  literature  under  the  title 
of  Buhl's  disease  or  acute  fatty  degeneration  of  the  newborn.  The 
cases  coming  under  this  title  occurred  in  children  born  at  term,  asphyc- 
tic for  some  unknown  reason,  and  often  dying  without  any  attempt  at 
respiration.  The  children  which  were  resuscitated  breathed  badly,  re- 
mained cyanotic,  hiemorrhages  appeared  in  the  skin  and  mucous  mem- 
branes, bloody  vomit,  bloody  fluid  evacuations  and  umbilical  ha?mor- 
rhages  occurred  and  the  infants  rapidly  died.  In  case  they  lived  on  for 
a  few  days  longer,  severe  anaemia,  icterus  and  sometimes  also  anasarca 
occurred  and  then  death  in  collapse. 

At  necropsy,  beside  countless  haemorrhages  in  the  organs,  there 
was  found  fatty  degeneration  of  the  cells  of  the  pulmonary  alveoli,  of 
the  muscles,  the  heart,  liver  and  the  cpithehum  of  the  uriniferous  tubules. 

The  disease  is  said  to  occur  sporadically  anil  to  closely  resemble 
the  disease  of  animals  known  as  "spring-halt,"  in  its  manifestations  and 
pathological  anatomy.  However,  Bollinger  proved  that  this  latter  dis- 
ease as  it  occurs  in  foals  is  rarely  an  umbilical  sepsis.  It  is  highly  prob- 
able that  the  symptom-complex  described  by  Buhl  is  nothing  else  than 
sepsis,  which  it  so  closely  resembles. 

The  clinical  picture  which  Winckel  described  as  icteric  cyanosis  or 
cyanosis  icterica  perniciosa  cum  haemoglobinuria  (also  known  as  epi- 
demic  htemoglobinuria),   resembles  sepsis  in   many   aspects.     Winckel 

II— 5 


(id  THE   DISEASES   OF   CHILDREN 

first  observed  this  disease  occurring  as  an  epidemic  in  the  maternity  at 
Dresden,  and  since  then  only  a  few  cases,  also  sporadic,  have  been 
reported. 

These  cases  occurred  in  vigorous  full-term  children  during  the  first 
days  of  life,  in  whom  the  principal  manifestation  was  a  yellow,  icteric, 
and  then  deep  blue  discoloration  of  the  entire  cutaneous  surface  and  of 
all  the  mucous  membranes,  just  as  with  the  severest  cyanosis;  and 
further  a  brownish  color  of  the  urine.  The  urine  contained  hajmoglobin, 
blood  corpuscle  casts,  renal  epithelium,  bacteria,  urate  of  ammonia,  and 
small  quantities  of  albumin.  The  children  had  little  or  no  fever,  rapid 
respiration  and  died,  sometimes  in  convulsions.  The  blood  was  mark- 
edly thickened,  contained  numerous  granules  and  a  slight  increase  in 
the  number  of  leucocytes. 

At  necropsy  besides  the  icterus,  countless  hsemorrhages  were  found, 
especially  in  the  mucous  and  serous  membranes;  also  fatty  degenera- 
tion of  the  organs  and,  especially  characteristic,  masses  of  granular 
haemoglobin  in  the  kidneys  and  spleen.  In  all  cases,  with  one  excep- 
tion, the  umbihcal  vessels  were  found  to  be  healthy. 

In  a  siinilar  epidemic  reported  by  Kamen,  in  which,  however, 
hfemoglobinuria  was  absent,  a  bacillus  was  found  in  the  organs  and 
blood,  which  Kamen  considered  identical  with  the  bacillus  coli  com- 
munis. Kamen  found  the  same  bacillus  in  well-water;  according  to  his 
opinion  the  children  were  infected  through  cleansing  the  mouth.  After 
the  well  had  been  closed  up  the  epidemic  ceased.  As  Epstein  has  already 
assumed,  Winckel's  disease  is  also  nothing  more  or  less  than  sepsis. 

The  classifying  of  these  cases  under  a  separate  head  is  hardly  justi- 
fied, as  no  proof  has  been  furnished  of  the  presence  of  a  htemoglobinuria 
and  moreover  the  presence  of  red  blood  corpuscle  casts  argues  against  a 
hiemoglobinuria;  the  finding  of  lirown  granules  in  the  urine  and  kid- 
neys is  of  no  import  since  no  chemical  examination  was  made  to  deter- 
mine whether  or  not  they  were  composed  of  blood-pigment. 

Symptoms. — 1.  Fever.  This  is  not  a  constant  manifestation.  A  rise 
in  temperature,  at  times  very  high,  occurs  at  the  onset  in  the  majority 
of  cases.  The  further  coui'se  is  then  either  afebrile  or  else  attended  by  a 
very  irregular  fever.  Chills  never  occur.  Toward  the  end  of  the  disease 
a  markedly  subnormal  temperature  is  the  rule. 

2.  Sensation  is  benumbed  in  many  cases;  the  children  are  at  times 
comatose  and  at  times  show  great  unrest,  jactitation,  tremors  and  pro- 
longed, severe,  unvarying  cries  which  are  the  expression  of  a  state  of 
excitation;  this  state  sometimes  alternates  with  profound  apathy  in 
which  the  reflexes  are  diminished  and  may  be  totally  absent.  Convul- 
sions are  rare.  Upward  rotation  of  the  Ijulbi  with  open  eyes,  as  in 
sleep  and  flaccidity  of  the  muscles  occur;  but  more  often  p.seudotetanic, 
spastic  conditions  of  the  muscles  of  the  extremities,  trunk,   neck  and 


DISEASES    OF    THE    NEWBORN  67 

head;  with  trismus,  rigidity  of  the  neck  and  extreme  flexion  of  the  hand 
and  finger-joints,  which  is  more  frequent  the  younger  the  chihl.  Paral- 
yses do  not  belong  to  the  usual  picture  of  sepsis  and  depend  on  compli- 
cations with  meningitis  or  encephalitis. 

3.  The  skin  presents  varying  signs.  Children  in  the  first  weeks  of 
life  usually  sliow  a  marked  icterus,  which  may,  especially  witli  umbili- 
cal sepsis,  attain  the  degree  of  a  bronzing  fPorak  and  Durante).  Older 
infants  have  often  a  livid,  ashen-gray  color  which  easily  becomes  bluish 
(cyanosis)  on  the  distal  parts  of  the  body  and  on  the  mucous  membranes. 
There  is  often  iTdema  of  the  feet,  over  the  tibia^  and  in  the  pubic  region. 
In  some  instances  sclercedema  and  more  rarely  sclerema  occur  toward 
the  end  of  the  disease,  especially  in  premature  children,  during  the  first 
months.  The  hannorrhages  are  characteristic  and  very  important 
diagnostically;  they  spread  over  the  trunk  and  extremities,  sometimes 
as  small  petechise  and  sometimes  as  more  or  less  extensive  effusions. 
Furuncles  and  extensive  skin  abscesses — as  a  rule  not  embolic  but 
occurring  through  infection  from  without — are  frequent  findings  and 
also  often  form  the  entrance  point  for  the  infection.  Bed  sores  occur  not 
uncommonly  over  the  parietal  bones,  the  sacrum,  the  heel,  the  internal 
malleoli  and  more  rarely  the  elbow;  the  sores  soften  and  lead  to  deep 
ulcerations  which  sometimes  extend  to  the  periosteum  and  cause  necro- 
sis of  the  bones.  Erysipelas  is  not  rarely  added  to  the  above,  especially 
in  cases  of  umbilical  sepsis.  The  various  septic  erythemata  are  charac- 
teristic; they  occur  at  times  as  small  rose  .spots,  at  times  as  a  diffuse' 
redness,  seldom  similar  to  erythema  multiforme  and  only  exceptionally 
as  an  urticaria;  their  tendency  is  to  spread  over  the  trunk  and  extrem- 
ities. At  times  blebs,  \nth  purulent  or  serous,  rarely  liloody.  contents, 
are  formed. 

4.  The  mucous  membrane  of  the  mouth  is  very  often  loosened,  espe- 
cially in  young  infants,  and  not  rarely  covered  with  haemorrhages. 
Fissures  in  the  corners  of  the  mouth,  catarrhal  inflammations  with  or 
without  thrush,  processes  leading  to  necrosis  of  the  mucosa,  which  are 
sometimes  followed  by  necrosis  of  the  jaw-bones;  inflammations  and 
suppurations  of  the  salivary  glands  (especially  the  parotid)  which  on 
pressure  fill  the  mouth  -n-ith  pus;  all  these  are  among  the  most  frequent 
occurrences.  Catarrhal  rliinitis  sometimes  causes  profuse  purulent  dis- 
charge with  the  formation  of  fissures  on  the  alse  nasi  and  more  rarely 
severe  epistaxis.  In  some  cases  the  fissures  or  the  inflamed  or  necrotic 
mucous  membranes  bleed,  thus  at  times  leading  to  the  diagnosis  of 
meliEna. 

5.  The  eyes  are  not  usually  involved  in  the  systemic  infection. 
Retinal  haemorrhages  have  been  found  repeatedlj-  in  the  newborn  and 
are  of  value  diagnostically.  Ha-morrhages  from  the  hds,  at  times  uncon- 
trollable, are,  according  to  Ritter,  of  septic  origin  in  the  majority  of 


68  THE   DISEASES   OF   CHILDREN 

instances  in  which  there  is  no  primary  local  disease  (blennorrhcea,  etc.). 
Otitis  media  occurs  very  often;  but  extension  of  the  process  to  the  bones, 
sinuses,  brain  or  meninges  belongs  to  the  rarest  complications. 

6.  The  respiratorij  organs  are  most  severely  involved  in  the  symp- 
tom-complex of  sepsis.  Even  in  uncomplicated  cases  dyspnoea  with 
very  rapid  breathing  is  present  as  an  index  of  a  severe  intoxication. 
Bronchitis  and  pneumonia,  which  are  only  demonstrable  when  some- 
what extensive;  multiple  abscesses  of  the  lungs,  clinically  not  demon- 
strable; serofibrinous  and  purulent  pleurisies,  all  belong  to  the  most 
frequent  occurrences.  When  they  form  the  only  salient  clinical  feature 
the  disease  is  termed  "septic  infection  with  pulmonary  symptoms" 
(Fischl). 

7.  Pericarditis  occurs  cjuite  often,  as  an  involvement  of  the  circu- 
latory apparatus,  in  consequence  of  the  general  infection.  It  usually 
arises  by  extension  from  the  pleura,  or  more  rarel}'  from  the  medias- 
tinum. Endocarditis  has  been  observed  several  times,  on  the  whole, 
however,  rarely.  In  the  diagnosis  of  this  complication  in  infants  we 
often  encounter  insurmountable  difficulties  (Finkelstein).  The  behavior 
of  the  pulse  is  in  no  way  characteristic.  Just  as  in  all  severe  diseases  of 
the  infant,  cardiac  weakness  easily  comes  on  and  either  leads  acutely 
to  death  or  else  persists  for  days,  accompanied  by  a  subnormal  body 
temperature. 

8.  The  gastro-intestinal  canal  is  very  often  involved  in  the  clinical 
picture  of  sepsis.  Vomiting  and  diarrhoea  are  at  times  the  only,  often 
the  most  striking  symptoms.  The  sepsis  not  uncommonly  runs  its 
course  from  the  very  beginning  under  the  clinical  picture  of  a  gastro- 
intestinal catarrh;  more  frequently,  however,  severe  diarrha?as  occur 
during  the  course  of  sepsis,  and,  what  is  worthy  of  note,  in  artificially 
fed  as  well  as  breast-fed  infants  (Ritter  et  al.). 

The  vomitus  is  bile  stained  in  some  instances,  in  others  it  is  col- 
ored from  blood-red  to  brownish,  the  stools  are  fluid  or  soft,  yellow  or 
green  and  often  there  is  an  admixture  of  blood,  brown  or  blackish 
brown  in  color,  rarely  dark  red  (mehTua).  The  abdomen  is  distended 
very  frequently  and  the  peristalic  action  very  lively;  at  times  intestinal 
paralysis  with  tremendous  distention  of  the  abdomen  occurs,  pushing 
up  the  diaphragm  and  causing  dyspnoea;  purulent  peritonitis  occurs 
very  often,  especially  in  cases  of  uml^ilical  sepsis.  Recognition  of  the 
peritonitis,  however,  is  difficult;  fluid  exudate  is  usually  not  present  in 
demonstrable  quantities.  The  presence  of  considerable  tenderness  over 
the  abdomen  may  be  considered  as  evidence  of  peritonitis. 

9.  The  spleen  is  frequently  enlarged,  but  the  swelUng  is  of  no  value 
diagnostically,  as  it  occurs  in  the  nursling  in  so  many  other  conditions. 

10.  The  liver  seldom  shows  any  clinical  signs  except  the  icterus,  the 
import  of   which  has  already  been  discussed.     The  edge  of  the  liver  is 


DISEASES    OF    THE    NEWBORN  C!) 

palpable  in  the  healthy  nursling  when  the  abdomen  is  free  from  disten- 
tion; therefore,  the  fact  that  the  liver  is  palpable  should  be  used  cau- 
tiously for  diagnostic  purposes. 

11.  The  urine  usually  contains  albumin,  rarely  sugar  (milk-sugar). 
The  albuminuria  is  either  an  expression  of  a  toxic  degeneration  of  the 
kidneys  or  of  a  true,  rarely  ha?morrhagic,  nephritis  or  a  pyelocystitis. 
In  children  of  the  first  weeks  of  life  who  have  had  icterus,  bile-pigment  is 
often  found  in  the  urine  (see  chapter  on  icterus  neonatorum).  In  many 
cases  haemoglobin  in  solution  and  in  pigment  granules  has  been  found 
(Winckel's  disease).  The  urinary  sediment  should  be  examined  in 
every  case  and  gives  findings  corresponding  to  the  involvement:  hya- 
Hne,  epithehal  or  granular  casts,  casts  of  blood  corpuscles,  pus  cells, 
either  isolated  or  in  clumps,  epithelial  cells  and  leucocytes. 

The  sexual  organs  as  a  rule  are  normal,  although  Ritter  reports 
genital  haemorrhages  and  vaginal  catarrh  occurring  during  the  course  of 
septic  infection  in  the  newborn. 

12.  The  bones  and  joints  show  severe  changes  in  occasional  cases; 
periostitis  and  osteomyelitis  occur  rarely  in  the  infant;  multiple  inflam- 
matory foci  in  the  joints,  especially  the  hip  and  shoulder  joints  (Czerny 
and  Moser)  betray  their  existence  by  the  immobility  and  the  oedema  of 
the  affected  extremity  and  more  rarely  by  a  reddening  over  the  joint. 
Pressure  over  the  diseased  area  and  passive  movement  of  the  affected 
extremity  elicit  expressions  of  pain.  AVith  appropriate  passive  motion 
at  the  ends  of  the  bones  one  is  often  able  to  elicit  a  fine  crepitation,  a 
sign  of  epiphyseal  separation,  which  does  not  occur  with  hereditary 
syphilis  alone. 

13.  The  blood  may  show  startling  changes.  A  decrease  in  the  num- 
ber of  erythrocytes  is  frequent;  a  polynuclear  leucocytosis  has  been 
found  more  rarely.  Noteworthy  is  the  deficient  coagulability  of  the 
blood,  which  must  be  attributed  to  the  action  of  toxins  and  reminds  one 
of  the  behavior  of  the  blood  when  peptone  and  certain  other  poisons 
(leech  extract,  etc.)  have  been  injected.  In  some  cases  (Winckel's  dis- 
ease) the  blood  was  "syrup  thick,"  and  microscopically  countless  blood 
granules,  due  to  destruction  of  the  red  cells,  were  found.  In  other  cases 
hemoglobin  and  also  methsemoglobin  were  demonstrated  in  the  serum 
with  the  spectroscope. 

Anatomical  Findings. — The  pathological  changes  found  in  sepsis  of 
the  newborn,  with  the  exception  of  certain  characteristics  due  to  the 
pecuHar  behavior  of  certain  organs  in  infancy,  are  the  same  as  those  in 
sepsis  of  the  adult.  In  the  acute  cases  and  those  without  metastatic 
abscesses  there  are  two  principal  lesions  regularly  found;  namelj^ 
haemorrhages  and  parenchymatous  degeneration  of  the  heart  muscle, 
liver  and  kidneys.  The  hsemorrhages  are  scattered  throughout  practi- 
cally   all    the   organs;  aside   from    the   clinically    demonstrable   luemor- 


70  THE   DISEASES   OF   CHILDREN 

rhages  in  the  skin,  mucous  membranes  and  retina,  they  are  found  regu- 
hirly  in  the  dura,  sometimes  in  the  pia  and  more  rarely  in  the  brain 
substance. 

The  findings  in  the  various  organs  agree  and  vary  with  the  chnical 
symptoms  and  there  will  be  mentioned  only  those  results  of  pathological 
examination  to  which  no  reference  has  been  made  in  discussing  the 
clinical  manifestations. 

The  brain  is  often  oedematous  and  hypertemic;  the  meninges  are 
congested  and  scattered  with  hiemorrhages.  Haemorrhages  in  the  brain 
substance  itself  are  not  so  common;  encephalitis  and  meningitis  have 
been  observed  at  times,  brain  abscesses  rarely.  Foci  of  lobular  pneumo- 
nia are  very  often  demonstrable,  as  also  atelectatic  areas,  small  multi])le 
embohc  abscesses  and  frequently  also  infarctions.  Fatty  degenera- 
tion of  the  alveolar  epithelium  is  not  uncommon  in  the  form  of  sepsis 
described  as  Buhl's  disease.  The  pleurae  are  often  the  seat  of  inflam- 
mation, usually  serofibrinous  or  purulent.  The  heart  muscle,  in  conse- 
cjuence  of  the  intoxication,  shows  parenchymatous  or  fatty  degenera- 
tion; it  is  pale  and  often  shows  the  so-called  tiger  markings;  the  valves 
are  only  rarely  the  seat  of  an  inflammatory  process,  the  pericardium 
much  more  frequently.  The  liver  usually  shows  parenchymatous  degen- 
eration. Not  uncommonly,  as  the  result  of  an  umbilical  phlebitis,  we 
have  multiple  abscesses  in  the  liver,  which  in  Hfe  give  rise  to  no  symp- 
toms. The  spleen  shows  signs  of  acute  or  chronic  swelling.  The  gastro- 
intestinal tract  may  be  found  either  normal  or  slightly  inflamed.  In 
occasional  cases  we  have  a  pronounced  acute  gastro-enteritis  and  in 
these  cases  the  mesenteric  lymph-glands  are  found  considerably  swollen. 
Haemorrhages  are  found  very  commonly  in  the  mucosa  as  well  as  in  the 
serosa  of  the  stomach  and  the  entire  intestinal  canal.  The  peritoneum 
is  the  seat  of  an  acute  inflammation  practically  only  in  cases  of  umbili- 
cal sep.sis,  otherwise  it  is  normal.  The  kidneys  show  pathological 
changes  in  every  case;  at  times  only  a  parenchymatous  degeneration 
and  at  times  fatty  degeneration  with  necrosis  of  the  renal  epithehum; 
hipmorrhages,  round-cell  infiltrations,  interstitial  inflammatory  changes 
and  diseases  of  the  pelvis  are  the  findings  corresponding  to  the  varying 
involvement  of  the  urinary  apparatus  in  the  septic  process.  Even  in 
the  milder  cases  we  very  often  find  haemorrhages  in  the  bone  marrow 
and  sometimes  metastatic  periostitis,  osteomyehtis  and  arthritis;  the 
latter  with  cither  .serous  or  purulent  effusion.  At  the  umbilicus  not  un- 
commonly we  find  the  changes  described  in  the  chapter  on  diseases  of 
the  umbilicus. 

The  diagnosis  of  sepsis  must  in  general  be  considered  as  difficult. 
Bacteriological  examination  of  the  blood  furnishes  the  most  rehable  re- 
sults. Inasmuch,  however,  as  it  is  always  necessary  to  draw  off  consid- 
erable quantities  of  blood  and  as  this  can  only  be  done  unimpeachably 


DISEASES    OF   THE    NEWBORN  71 

by  puncture  or  incision  of  a  vein  and  not  from  the  ball  of  a  finger  or  toe, 
it  is  evident  that  this  examination  can  only  be  carried  out  in  a  small 
percentage  of  infants  during  Hfe.  Added  to  this  is  the  fact  that  bacteria 
are  not  found  in  the  blood  in  life  in  a  considerable  percentage  of  cases 
of  sepsis.  It  is  recommended  to  make  the  blood  examination  during 
the  death  agony  or  immediately  post  mortem,  a  method  of  procedure 
which  can  usually  be  carried  out  with  infants.  Sometimes  the  bacteria 
may  be  found  in  numbers  in  co\'er-slip  preparations  from  the  l)lood  of 
the  cadaver.  In  some  cases  information  may  be  furnished  by  puncture  of 
organs  which  are  the  seat  of  metastatic  inflammations  and  the  bacterio- 
logical examination  of  the  body  fluids,  only  rarely  the  bacteriological 
e.xamination  of  the  urine  which  is  drawn  by  catheter. 

In  the  majority  of  cases,  however,  we  must  get  along  without  bac- 
teriological examinations  of  the  blood  (lumbar  puncture  furnishes  no 
certain  evidence,  since  bacteria  are  found  in  the  cerebrospinal  fluid  in 
the  absence  of  sepsis,  under  certain  conditions).  Then  the  diagnosis 
must  be  made  from  the  clinical  information.  When  the  primary  focus 
is  demonstrable,  when  the  infants  have  fever,  and  metastases  or  haemor- 
rhages show  themselves,  we  are  probably  justified  in  making  the  diag- 
nosis. The  examination  of  the  retina,  recommended  by  Herrenheiser 
and  Fischl,  may  be  of  value  diagnostically.  However,  we  must  not 
lose  sight  of  the  fact  that  in  the  newborn  retinal  haemorrhages  may  also 
be  caused  by  birth  trauma.  In  some  cases  it  is  impossible  in  the  absence 
of  bacteriological  anatomical  evidence  to  decide  whether  or  not  we  are 
dealing  with  sepsis.  In  acute  cases  sepsis  must  be  differentiated  from 
acute  gastro-enteritis  and  pneumonia,  and  in  children  only  a  few  days 
old,  from  atelectasis  and  cerebral  or  meningeal  hemorrhage;  the  cases 
running  a  subacute  or  chronic  course  must  often  be  differentiated 
from  chronic  nutritional  disturbances  carrying  secondary  diseases  in 
their  train. 

The  prognosis  is  very  serious;  the  more  acute  the  course  of  the  dis- 
ease, the  younger  and  more  immature  the  affected  infant  and  the  more 
organs  e^^dently  involved,  the  more  unfavorable  the  outlook. 

Prophylaxis  and  Treatment. — Since  sepsis  occurs  especially  where 
children  are  cared  for  in  numbers,  as  in  institutions,  it  behooves  us  to 
employ  all  those  means  which  have  been  so  effective  in  the  prevention 
of  puerperal  fever.  The  strictest  asepsis  in  the  care  of  the  newborn  is 
enjoined.  The  care  of  the  child  from  the  moment  of  birth  should  not  be 
assigned  to  the  same  attendant  caring  for  the  puerperal  woman.  The 
umbilical  dressing  must  be  aseptic,  the  oral  cavity  should  not  be  cleansed : 
the  clothing  of  the  infant  should  be  sterilized  and  the  hands  of  the  atten- 
dant clean  and  disinfected  when  changing  the  clothes.  All  utensils 
should  be  individual  for  each  child  and  should  be  kept  sterile  (bath-tub, 
basins,   cotton,   thermometer,  etc.).     In  hospitals  the  isolation   of  all 


72  THE   DISEASES   OF   CHILDREN 

nurslings  is  of  special  value  (the  box  system  of  Grancher,  or,  still  better, 
isolation  cells  or  rooms).  For  the  breast-feeding  of  infants,  the  breast 
should  be  carefully  cleansed  before  each  nursing  and  in  artificial  feeding 
the  strictest  asepsis  of  food,  bottles  and  nipple  must  be  carried  out. 
Heubner  has  recommended  that  in  institutions  the  feeding  of  children 
should  not  be  entrusted  to  the  attendants  in  charge  of  the  cleansing  of 
the  children. 

In  the  care  of  premature  children,  kept  in  incubators,  it  becomes 
unqualifiedly  necessary  to  strive  for  strict  disinfection  of  the  incubator 
and  a  sufficient  supply  of  clean  air. 

The  treatment  of  the  developed  disease  is  purely  symptomatic. 
Together  with  the  combating  of  the  individual  symptoms  one  must  de- 
vote attention  to  the  care  of  the  skin  and  the  nourishment;  artificially 
fed  children  should  be  furnished  with  human  milk  since  through  this 
the  chances  of  recovery  are  increased.  All  abscesses  must  be  opened  and 
treated  according  to  surgical  principles.  Bandages  should  be  limited 
as  much  as  possible  in  order  that  healthy  areas  of  the  skin  may  not  be 
involved  through  the  bandage.  The  heart's  action  should  be  carefully 
supported;  one  may  employ  strong  tea,  small  doses  of  digitahs  (0.05  Gm. 
(4  gr-)  pro  die  in  infusion)  or  digalen  |  c.c.  (n\,  TJ)  internally  daily, 
cafTein  (caffein  sodium  saHcylate  0.05  to  0.1  Gm.  (|  gr.-li  gr.)  pro  die)  or 
camphorated  oil  0.3  to  0.5  c.c.  (ni  4-8)  subcutaneously  (possibly  two  to 
three  times  daily).  The  subcutaneous  injection  of  physiological  saline 
solution  (nine-tenths  of  1  per  cent.)  in  amounts  20-50  Gm.  (11-28  drams), 
according  to  age,  is  recommended  by  many.  Just  as  with  adults,  col- 
loidal silver  may  be  tried  and  especially  per  rectum  or  by  intravenous 
injection  of  0.1  Gm.  (Ih  gr.)  (Finkelstein).  The  use  of  specific  immune 
sera  has  up  to  the  present  time  shown  no  beneficial  results. 

V.     MEL.ffiNA  NEONATORUM 

The  name  mela>na  neonatorum  is  not  appfied  to  any  one  disease  as 
an  entit}'.  It  rather  serves  to  cover  a  symptom-complex,  the  loss  of 
blood  from  the  gastro-intestinal  tract,  either  from  the  stomach  by  vom- 
iting, or  from  the  rectum,  or  both.  One  is  justified  in  expunging  melsena 
from  the  category  of  diseases  of  the  newborn.  However,  inasmuch  as 
loss  of  blood  from  the  gastro-intestinal  canal  often  forms  the  most 
marked  and  at  times  the  only  demonstrable  symptom  in  certain  mala- 
dies of  the  newborn,  we  give  this  symptom-complex  special  considera- 
tion. We  will  endeavor,  therefore,  to  give  a  brief  description  of  this 
symptom-complex  and  its  consequences  and,  by  comparing  its  clinical 
picture  with  the  hitherto  known  anatomical  findings,  to  show  the  method 
by  which  the  examining  physician  can  make  a  diagnosis  of  the  under- 
lying disease.  We  must  again  emphasize  the  statement  that  a  diag- 
nosis of  melgena  is  unjustifiable  and  that  endeavor  must  be  made  in 


DISEASES    OF    THE    NEWBORN  73 

every  instance  to  discover  the  disease  responsible  for  the  hirmatoniesis 
or  tlie  hteniorrliage  in  the  intestinal  canal.  There  are  thi-ee  varieties  of 
melaena, — spurious,  symptomatic  and  true.  Spurious  mela'na  desig- 
nates the  loss  of  blood  from  the  gastro-intestinal  tract  when  the  hienior- 
rhage  does  not  really  originate  in  the  mucosa  of  the  gastro-intestinal 
tract.  Thus  we  know  that  epistaxis  frequentl}^  causes  the  vomiting  of 
blood  in  the  newborn.  The  epistaxis,  moreover,  is  often  overlooked, 
because  the  blood  instead  of  escaping  from  the  anterior  nares  runs  back- 
ward into  the  pharynx  and  a'soi)hagus  and  thence  into  the  stomach.  In 
such  cases  one  is  occasionally  able  to  see  streaks  of  fresh  blood  on  the 
posterior  pharyngeal  wall  (Swoboda).  This  epistaxis  in  children,  how- 
ever, depends  upon  various  pathological  processes  in  the  nasal  mucosa: 
syphilitic  rhinitis,  ulcerations  with  septic  processes,  nasal  diphtheria 
(observed  in  syphihtic  children,  Swoboda).  In  still  other  cases  the 
blood  comes  from  the  mucosa  of  the  lips  or  mouth.  Ulcers  whose  seat 
of  predilection  is  the  angle  of  the  mouth,  Bednar's  aphthte  and  stoma- 
tides,  especially  such  as  tend  to  necro.sis,  must  be  taken  into  considera- 
tion. In  other  cases  spurious  niehena  is  due  to  wounds  of  the  buccal 
mucosa;  such  wounds  may  be  caused  during  delivery  by  the  finger  of 
the  accoucheur;  or  later,  in  the  bloody  separation  of  an  adherent 
fra!nuni  of  the  tongue,  an  operation  which  was  formerly  extensively 
performed. 

It  could  only  be  under  the  most  complicated  conditions  that  a  pul- 
monary hemorrhage  in  the  newborn  would  lead  to  the  vomiting  of 
blood.  However,  pulmonary  hjemorrhages  have  been  observed  in  the 
newborn  (Billard,  Barthez  and  Rilliez,  and  recently  by  Esser),  but  to 
my  knowledge  hematemesis  is  not  mentioned  in  the  report  of  these 
cases,  all  of  which  ran  an  extremely  rapid  course  without  cough.  How- 
ever in  older  cliildren  the  passage  of  blood  from  the  st  )mach  or  intes- 
tines may  occasionally  be  observed  with  pulmonary  haemorrhage. 

Not  infrequently  the  haemorrhage  does  not  originate  in  the  cliild  at 
all  but  in  the  mother.  Fissures  of  the  maternal  nipple  bleed  during  the 
act  of  nursing  and  the  newborn  swallows  maternal  blood,  wliich  at  times 
is  vomited  or  colors  the  feces  black.  In  such  cases  however,  there  can 
be  but  a  scanty  admixture  of  blood.  In  some  cases  spurious  melcena  is 
attributed  to  the  swallo-R-ing  of  blood  during  birth,  for  example,  with 
premature  detachment  of  the  placenta.  In  case  spurious  mela-na  can 
be  excluded,  one  then  has  to  deal  with  a  haemorrhage  originating  in  the 
blood  vessels  of  the  gastro-intestinal  tract.  ^Ye  speak  of  symptomatic 
melcena  when  the  gastro-intestinal  ha?morrhage  forms  only  one  symptom 
of  a  demonstrable  general  disease;  of  true  mela'na  when  the  haemorrhage 
and  its  consequences  dominate  the  entire  disease  picture.  It  is  self- 
e\adent  that  a  strict  alignment  into  one  of  the  above  classes  is  often 
impossible. 


74  THE   DISEASES   OF   CHILDREN 

We  shall  discuss,  first,  the  cause  of  such  a  ha'inorrhage  and  then  the 
diseases  and  conditions  that  can  be  considered  responsible  for  its  etiology. 

Course. — Htrmorrhage  from  the  gastro-intestinal  tract  usually 
comes  on  within  the  first  four  days  of  life,  most  often  on  the  first  and 
second  day,  rarely  later.  In  Silbermann's  collected  report  of  42  cases, 
tlie  htemorrhage  commenced  on  the  first  day  in  eleven  cases  and  on  the 
second  day  in  sixteen  cases.  Evidence  of  the  bleeding  is  most  often 
seen  in  the  stools;  sometimes  the  meconium  is  streaked  with  blood  or 
covered  with  clots,  or  we  find  a  blood-red  halo  around  the  stool  on  the 
diaper.  The  bloody  evacuation  is  usually  trifling  at  first,  but  often 
increases  in  amount  so  that  the  stool  is  made  up  entirely  of  large  clots 
or  else  is  liquid,  dark  red  or  almost  black  in  color  and  contains  small 
solid  particles.  In  some  cases  (according  to  Silbermann,  23  per  cent.) 
the  intestinal  bleeding  is  accompanied  by  ha>matemesis,  in  the  minority 
(16  per  cent.)  ha-mateme-sis  forms  the  only  symptom.  In  these  latter 
cases  the  child  vomits  a  dark  red  or  brown  fluid  iu  which  there  are  many 
small  clots.  In  case  the  child  has  already  ingested  milk,  black  or  brown 
lumps  are  vomited,  together  with  the  discolored  milk,  shortly  after  the 
feeding.  Sometimes,  however,  there  are  onlj-  small  streaks  of  blood 
demonstrable  in  the  vomitus. 

Ha^matemesis  and  bloody  stools  may  cease  after  a  single  occurrence; 
then,  if  no  underh'ing  disease  be  present,  the  child  will  show  no  signs  of 
chsease.  In  other  cases,  however,  the  blood}'  vomit  and  stools  recur  and 
then  the  ine"vdtable  consequences  of  severe  loss  of  blood  become  evident; 
the  temperature  falls,  the  distal  parts  of  the  body  become  cooled  off, 
the  face  takes  on  a  waxen  pallor,  the  pulse  becomes  hardly  perceptible, 
the  heart  sounds  weak  and  the  respiration  superficial;  there  is  suj)- 
pression  of  urine  and  refusal  to  take  nourishment.  Rarely  there  are 
convulsions.  In  case  the  bleeding  stops,  the  infant  may  recover  even 
in  severe  cases,  otherwise  it  succumbs  as  a  result  of  the  loss  of  blood, 
or,  recovering  from  this,  dies  from  the  underlying  disease. 

It  is  self-exddent  that  the  symptoms  are  appreciably  different  when 
we  are  dealing  with  a  constitutional  disease  which  has  given  rise  to 
.sjMnptomatic  niehena.  Sometimes  the  bleeding  lasts  for  only  a  few  hom-s 
and  sometimes  for  several  days. 

Causes  of  Gastro-intestinal  Haemorrhage. — Formerly,  mela^na,  like 
other  hicmorrhages  in  the  newborn,  was  attributed  to  a  "hEemophiha." 
This  view,  however,  is  either  not  tenable  at  all,  or  else  it  holds  good  for 
only  the  rarest  of  cases.  In  the  first  place  hsemophilia  usually  does  not 
occur  during  the  first  months  of  life;  secondly,  in  children  that  have 
recovered  from  meUena,  the  "haemophilia"  also  disappears;  further, 
hsemophiha  is  a  disease  which  generally  affects  males:  and,  lastly,  it  is  an 
hereditary  disease  transmitted  through  the  mother.  All  these  are  rea- 
sons which,  with  a  careful  history,  permit  as  a  rule  of  the  exclusion  of 


DISEASES    OF    THE    NEWBORN  75 

hjeniophilia.  It  was  thought  necessary  to  assume  htemophilia  wlieu 
several  cases  occured  in  the  same  family  (not  hereditary).  As  a  matter 
of  fact  it  is  rather  striking  that  there  are  families  in  which  several  chil- 
dren have  had  niehrna  and  died  of  it  during  the  first  days  of  life.  J. 
Fischer  reviewed  the  cases  already  reported  and  added  his  own  observa- 
tions occurring  in  the  first  two  children  of  a  fanuly;  he  consitlered  htemo- 
phiha  as  the  etiological  factor  in  these  cases.  Proof  of  heredity,  how- 
ever, was  not  adduced;  a  third  child  in  the  same  family  had  no  mehena, 
Hke\A'ise  no  hiemophiUa.  We  must,  therefore  consider  the  assumption 
of  a  htemophiha  in  these  cases  as  entirely  unwarranted  and  admit  rather 
that  the  cause  of  melaena  in  such  cases  is  not  clear;  since  no  autopsy 
report  is  given  and  no  bacteriological  examination  was  made,  no  well- 
supported  opinion  concerning  the  origin  of  the  meUrna  can  be  given. 

In  other  cases  the  hiemorrhage  from  the  rectum  or  the  hsematemesis 
forms  only  a  part  of  the  symptom-complex  of  sepsis.  Septicaemia  often 
leads  to  haemorrhages;  this  has  already  been  shown  in  the  chapter  on 
sepsis.  Multiple  ha-morrhages  belong  typically  to  the  clinical  picture  of 
sepsis  of  the  infant;  however,  sepsis  not  infrequently  occurs  under  the 
guise  of  a  hsemorrhage  from  a  single  organ  and  then  sometimes  under 
the  clinical  manifestations  of  mehrna.  Klebs,  Ritter  and  Epstein  have 
shown  the  role  that  sepsis  plays  in  the  causation  of  haemorrhages  in  the 
infant  and  the  investigations  of  the  last  years  have  confirmed  the  opinion 
that  the  so-called  "  hsemorrhagic  diathesis  of  the  newborn,"  which 
leads  to  bleeding  in  various  organs,  is,  in  the  vast  majority  if  not  in  all 
cases,  nothing  other  than  septicannia.  The  investigations  of  Gartner, 
Neumann,  Babes,  Finkelstein  and  others  have  established  this.  More 
recently  endeavor  has  l:)een  made  to  attribute  melaena  neonatorum  to 
infection  ^ath  certain  specific  organisms.  Gartner  described  a  "  mela?na 
bacillus"  which  he  found  in  two  cases  of  mela>na.  Despite  the  lapse  of 
ten  years  since  his  communication,  no  confirmation  of  Ms  findings  is  at 
hand.  It  is  certainly  not  to  be  doubted  that  certain  species  of  bacteria 
are  particularlj'  prone  to  produce  lurmorrhages  with  the  infections  which 
they  cause;  but  the  specificitj*  of  neither  the  "mela-na  bacillus"  nor  the 
so-called  "bacillus  of  lia}morrhagic  infection  in  the  human"  (Babes)  has 
as  yet  been  established.  Moreover,  we  know  that  infection  with  the  most 
diverse  kinds  of  bacteria  can  lead  to  multiple  or  single  hai-morrhages. 

Next  to  sepsis  we  must  consider  hereditary  sypliiUs  as  an  important 
cause  of  gastro-intestinal  haemorrhage.  According  to  earher  reports 
(Behrend  and  others)  and  especially  according  to  the  thorough  investi- 
gations of  Mraczek,  hereditary  syphilis  brings  about  changes  in  the 
walls  of  the  small  and  medium  .sized  vessels  and  of  the  capillaries,  wliich 
lead  to  thickening  of  the  intima,  small-celled  infiltration  with  subse- 
quent connective-tissue  formation,  to  thickening  of  the  vascular  wall 
and  to  a  narrowing  and  even  occlusion  of  its  lumen.    Thus  venous  stasis 


76  THE   DISEASES   OF   CHILDREN 

and  h£emorrliagcs  ensue.  These  ha'morrhages  occur  also  in  the  intesti- 
nal canal  and  Mraczek  has  reported  such  cases  (Esser  also  recently  re- 
ported one  case).  The  assumption  that  these  were  cases  of  sepsis,  which 
develops  especially  easily  in  syphiUtic  children,  is  negatived  by  the  fact 
that,  in  some  of  Mraczek's  cases,  Kolisko  and  Paltauf  were  able  to  ex- 
clude sepsis  by  means  of  bacteriological  examinations.  In  the  vascular 
affections  of  hereditary  syphilis,  hsemorrhages  can  easily  occur  because 
of  stasis  and  disturbances  of  the  circulation,  and  at  times  syphilis  runs 
its  course  under  the  clinical  picture  of  melsena. 

The  gastro-intestinal  haemorrhages  which  Kundrat  described  occur- 
ring with  syphilis  of  the  liver  are  not  to  be  attributed  to  a  syphilitic 
affection  of  the  vessels  but  rather  to  abnormal  stasis  in  the  portal  circu- 
lation. However,  not  only  the  constitutional  diseases  just  spoken  of 
but  also  local  diseases  of  the  organs  may  lead  to  secondary  (sympto- 
matic) meltrna.  To  this  category  belong  first  and  foremost,  congenital 
anomalies  of  the  heart  and  large  vessels;  then  diseases  of  the  Uver,  e.g., 
syphilis  of  the  Uver,  which  Kundrat  advanced  as  the  cause  of  melfena; 
further,  tumors  of  the  abdomen  (cysts  in  Schukowski's  case),  then  local 
diseases  of  the  intestines  and  stomach,  which  lead  to  secondary  gastric 
hirmorrhage.  Cases  of  mehena  are  observed,  however,  with  compara- 
tive frequency,  in  otherwise  perfectly  healthy  children  (true  melscna). 
In  these  cases  the  haemorrhage  starts  ■within  the  first  four  days  of  life, 
usually  on  the  first  or  second  day.  In  the  cases  terminating  fatally,  no 
constant  findings  in  the  gastro-intestinal  tract  have  been  shown  at 
necropsy.  In  a  part  of  the  cases  nothing  which  could  have  explained 
the  haemorrhage  was  found;  in  others,  hypencmia  and  stasis  in  the 
gastro-intestinal  mucosa;  and  in  still  others,  multiple  ha?morrhages  or 
haemorrhagic  erosions  were  found  in  the  mucosa.  In  a  few  cases  a  small 
ulcer  similar  to  the  round  ulcer  of  the  adult,  was  found  in  the  stomach 
or  the  small  intestine  (duodenum)  or  very  rarely  in  the  oesophagus. 
Multiple  ulcers  were  rarely  present. 

Mela;na  is  most  easily  explained  in  the  cases  in  which  there  is  an 
ulcer  present.  The  erosion  of  a  blood  vessel  in  consecjuence  of  the  tissue- 
necrosis,  leads  to  bleeding  which  is  often  uncontrollable.  For  these 
cases,  however,  the  question  as  to  the  origin  of  the  ulcers  nuist  be 
raised;  Landau  advanced  the  hypothesis  that  emboli  gave  rise  to  them. 
The  emboli  are  supposed  to  originate  in  a  thrombosis  of  the  umbilical 
vein  or  of  the  ductus  arteriosus  Botalli;  the  thrombus  being  swept  into 
a  branch  of  the  pancreatico-duodenal  or  the  gastric  artery,  occludes  the 
vessel,  thus  causing  necrosis  and  ulceration.  The  gastro-intestinal 
bleeding  then  ensues  secondarily  when  the  vessel  is  involved  in  the 
tissue-necrosis. 

Kundrat  brought  forward  against  this  theory,  the  fact  that  Landau 
had  not  demonstrated  the  embolic  origin  of  the  ulcers;  that  in  one  of 


DISEASES    OF    THE    NEWBORX  77 

his  own  cases  no  embolism  existed  and  that  the  sweeping  of  an  embolus 
into  the  vessels  of  the  intestine  or  stomach  was  highly  improbable  be- 
cause the  position,  caUbre  and  course  of  these  vessels  are  verj^  unfavor- 
able for  the  lodgment  of  cmljoli.  It  is  much  more  probable  that  the 
gastro-duodcnal  ulcers  are  not  emljolic  in  nature;  they  j)robabl_v  develop 
through  hicmorrhages  into  the  nuicosa  by  reason  of  the  softening  and 
digestive  action  of  the  gastric  juice  on  these  infarctcd  areas  (Kundrat).- 
These  ulcers  are  usually  situated  in  the  duodenum,  less  often  in  the 
stomach  and  rarely  above  the  cardia  in  the  oesophagus.  Kundrat's 
conception  is  in  part  supported  by  a  case  of  Chrzanowski's,  in  which 
there  was  bloody  effusion  throughout  the  entire  thickness  of  the  oesoph- 
agus above  the  cardia  and  only  superficial  defects  were  demonstrable  in 
the  epitheUum.  Further,  the  many  cases  of  mehena,  in  which  superfi- 
cial hajmorrhagic  erosions  develop  on  a  mucosa  riddled  with  haemor- 
rhages, serve  to  substantiate  Kundrat's  position;  in  these  cases  the 
necrotic  process  has  not  progressed  deeply  enough  to  cause  ulcer.  That 
there  are  many  cases  in  which  hai'morrhages  alone  are  found  in  the 
mucosa  argues  to  this  effect.  In  short,  from  haemorrhage  to  erosion  and 
then  to  ulceration  we  have  only  a  series  of  pathological  changes,  the 
cause  of  which  must  lie  in  a  hypersemia  of  the  gastro-intestinal  mucosa. 
Moreover,  in  the  cases  in  which  no  change  has  been  found  in  the  gastro- 
intestinal tract,  we  must,  ■nath  Kundrat,  hold  that  such  a  hypersemia 
did  exist  during  life  which,  however,  at  necropsy  has  disappeared.  The 
cause  of  this  hypera>mia  is  not  clearly  established;  in  explanation 
many  theories  have  been  adduced.  A  hannorrhage  in  the  gastro-intes- 
tinal tract  can  take  place  only  under  two  conditions:  (1)  ^^ith  a  normal 
state  of  circulation  and  blood  pressure,  the  vessel  wall  being  rhseased 
and  thus  offering  inadequate  resistance  to  the  pressure  of  the  blood; 
(2)  with  normal  vessel  walls,  the  blood  pressure  either  local  or  general, 
being  abnormally  high.  Of  course  in  every  such  instance  ha>mateniesis 
does  not  necessarilj'  follow,  but  on  the  contrary  there  is  usually  only 
stasis  and  slight  hsemorrhage  in  the  substance  of  the  mucosa.  Naturally, 
these  two  factors  may  coexist  in  any  one  case. 

With  constitutional  diseases  like  syphilis,  sepsis  and  lurmophilia, 
we  assume  that  damage  to  the  vessel  walls  is  the  primary  and  causal 
factor.  In  children  otherwise  healthy,  however,  it  is  unwarranted  to 
assume  a  pathological  change  in  the  vessel  walls.  In  such  cases  we 
must,  therefore,  predicate  an  abnormal  stasis  in  the  vascular  system  of 
the  gastro-intestinal  tract,  which  leads  to  hypenvmia  and  haMiiorrhages 
in  the  mucosa  or  directly  to  the  outpouring  of  blood  on  its  surface. 

The  hypera-mia  and  stasis  can  in  turn  be  caused:  (1)  by  com- 
pression of  the  cord  during  labor;  (2)  by  post  partum  asphyxia;  (3) 
by  insufficient  respiration,  such  as  occurs  with  pulmonary  atelectasis, 
(4)  by  congenital  anomalies  of  the  heart  and  vessels;    (5)  by  cerebral 


78  THE   DISEASES   OF   CHILDREN 

and  meningeal  ha?morrhages;  (6)  by  local  circulatory  disturbances  in 
the  abdomen  (e.g.,  with  cirrhosis  of  the  liver).  It  is  well  estabUshed 
that  ha-morrhages  do  occur  in  the  various  organs  of  asphyctic  children, 
of  children  that,  in  consequence  of  some  obstruction  to  respiration, 
breathe  only  very  superficially.  But  it  has  not  yet  been  demonstrated 
that  cerebral  hivmorrhage  in  the  newborn  can  actually  give  rise  to 
•melsena.  The  interesting  experiments  instituted  by  Brown-Sequard, 
Schiff  and  Epstein  and  elaborated  by  Klosterhalfen,  were  carried  out 
on  young  animals  by  v.  Preuschen  and  Pomorski;  they  showed  that 
injuries  of,  and  hfemorrhages  into,  various  parts  of  the  brain  lead  to 
haemorrhages  in  the  gastro-intestinal  canal.  This  probably  depends 
upon  a  disturl^ance  or  a  paralysis  of  the  vasomotor  centre,  which  in 
turn  leads  to  atony  of  the  vessels,  stasis  and  haMuorrhages.  Therefore, 
since  v.  Preuschen  found  cerebral  haemorrhage  in  two  cases  of  melsena, 
he  considers  himself  justified  in  attributing  melajna,  in  a  part  of  the 
cases,  to  cerebral  ha?morrhage.  It  is,  however,  possible  that  the  cere- 
bral and  intestinal  htemorrhages  exist  coordinately,  both  being  pro- 
duced by  the  same  cause;  this  question  has  not  yet  been  clearly  settled. 
The  Diagnostic  Import  of  Gastrointestinal  Haemorrhage. — In 
every  case  of  mehrna  an  efiort  nmst  be  made  to  establish  its  cause. 
Spurious  melffna  is  rather  easily  excluded;  to  this  end  careful  inspection 
of  the  nose,  buccal  cavity  and  the  posterior  pharyngeal  wall  must  be 
made  in  order  to  exclude  these  as  the  source  of  the  blood.  Examination 
of  the  maternal  breast  gives  evidence  as  to  fissures  of  the  nipples.  In 
case  spurious  melsena  can  be  excluded,  we  must  assume  that  the  seat  of 
the  bleeding  is  in  the  gastro-intestinal  tract.  Then  attention  must  be 
given  to  the  signs  by  which  hereditary  syphihs  can  manifest  itself  in  the 
newborn  (see  chapter  on  hereditary  syphihs).  Should  there  be  neither 
clinical  nor  anamnestic  evidence  of  the  presence  of  syphihs,  we  must 
by  means  of  the  history,  endeavor  to  exclude  or  establish  a  family  and 
hereditary  ha>mophilia.  However,  in  every  case,  even  though  evidence 
for  the  existence  of  syphilis  or  hsemophilia  be  at  hand,  we  must  endeavor 
to  settle  the  question,  whether  sepsis  may  or  may  not  be  the  principal 
causal  factor.  Consideration  of  the  symptoms  of  sepsis,  as  detailed  iu 
the  preceding  chapter,  will  decide  for  us  in  such  instances  (in  doubtful 
cases,  the  diagnosis  might  possibly  be  cleared  up  by  means  of  venesec- 
tion and  the  aseptic  -withdrawal  of  1-2  c.c.  of  blood,  for  bacteriological 
examination).  The  post-mortem  examination  of  the  heart's  blood  has 
not  as  much  diagnostic  value  as  the  examination  during  life.  I  nuist, 
therefore,  take  issue  with  those  writers  who  align  under  the  category  of 
sep.sis  all  cases  in  wliich  bacteria  (especially  bacillus  coli  communis) 
have  been  found  in  the  heart's  blood  after  a  long  death  agony.  Even 
less  ground  exists  for  the  opinion  tliat  all  cases  of  mehrna  are  due  to 
sepsis. 


DISEASES  OF  THE  NEWBORN  79 

.Although  the  presence  of  the  above-mentioned  maladies  is  not  pre- 
cluded by  the  absence  of  evidence  for  their  presence,  still  search  must 
then  be  directed  elsewhere  for  the  cause  of  the  haemorrhage.  The  local 
diseases  of  the  abdomen  and  its  viscera  must  be  taken  into  considera- 
tion, as  must  also  the  duration  of  the  labor  and  tlie  condition  of  the 
child  post  partum.  A  prolonged  labor  might  have  given  rise  to  a  cere- 
bral haemorrhage.  At  times  much  may  be  learned  from  the  condition 
of  the  fontanelles;  a  bulging  of  the  fontanelles  with  convulsions  sug- 
gests meningeal  or  cerebral  haemorrhage.  Lumbar  puncture,  in  case 
there  be  a  justifiable  suspicion,  may  occasionally  confirm  the  diagnosis. 
Asphyxia  has  occurred  in  only  a  small  percentage  of  the  observed  cases 
(9  per  cent.  Silbermanu).  A  careful  history  in  reference  to  asphj'xia 
neonatorum  must  be  elicited,  since  this  can  undoubtedly  give  rise  to 
haemorrhages.  A  careful  examination  of  the  chest  and  observation  of 
the  respiration  \\ill  determine  atelectasis  or  congenital  cardiac  defects. 

The  age  of  the  child  at  the  time  of  the  onset  is  of  diagnostic  value. 
It  is  highly  improbable  that  sepsis  is  responsible  for  the  hiemorrhage  in 
case  it  begins  immediately  or  shortly  after  parturition  and  no  e\'idence 
is  at  hand  for  the  existence  of  a  congenital  infection  or  an  infection 
acquired  diu-ing  Ijirth.  On  the  other  hand,  the  diagnosis  of  sepsis  is 
highly  probable  when  the  bleeding  starts  after  the  fourth  day  of  life. 
Haemorrhages  occurring  in  the  second  week  of  life  or  later  (Hitter  saw 
such  cases  occurring  as  late  as  the  second  month)  may  well  be  regularly 
laid  at  the  door  of  sepsis. 

The  presence,  in  the  stool,  of  large  quantities  of  only  shghth^  al- 
tered blood,  is  of  diagnostic  import,  since  in  such  cases  spurious  mehrna 
is  less  probable  than  in  eases  in  which  comparatively  small  amounts  of 
blood  are  vomited. 

There  still  remain  some  cases,  however,  which'in  spite  of  the  most 
thorough  investigation  are  explained  by  neither  the  clinical  examina- 
tion nor  the  post-mortem  findings  (at  the  necropsj'  careful  attention 
must  be  paid  to  the  nose  and  brain  and  to  blood  infection). 

The  prognosis  of  gastro-intestinal  hiemorrhage  varies  ^\'ith  that  of 
uuderljing  disease.  Silbermann  estimates  the  average  mortality  to  be 
44  per  cent.;  in  individual  cases,  where  organic  disease  can  be  excluded, 
the  prognosis  becomes  more  favorable   the  less  blood  the  child  loses. 

Individual  experience  is  hardly  adequate  in  this  connection,  since 
melaena  neonatorum  is  but  rarely  encountered.  My  own  observations 
are  limited  to  three  cases:  in  two,  sep.sis  was  the  cau.se  and  both  term- 
inated fatally;  the  third  case,  in  which  the  cause  was  undiscoverable, 
made  a  complete  recovery. 

However  the  very  numerous  pubUcations  of  later  years,  concern- 
ing the  results  of  gelatine  therap}-,  seem  to  promise  a  brighter  prognosis. 
Since  gelatine  does  not  cure  sepsis,  the  results  of  its  therapeutic  exhibi- 


80  THE   DISEASES   OF   CHILDREN 

tion  argue  for  the  opinion  that  only  a  part  of  the  cases  of  melsena  are  to 
be  attributed  to  sepsis. 

Treatment  can,  of  course,  avail  in  only  a  part  of  the  cases  of  meltena. 
The  best  results  have  followed  the  use  of  gelatine.  Merck's  absolutely 
sterile  (otherwise  danger  of  tetanus)  10  per  cent,  gelatine  is  injected 
subcutaneously,  under  aseptic  precautions,  10-25  c.c.  (2-6  drams)  and 
possibly  2-3  times  daily.  The  needle  wound  is  covered  with  gauze  and 
sealed  \vith  adhesive  or  collodion.  At  the  same  time  gelatine  may  be 
given  by  the  mouth,  in  a  2-5  per  cent,  solution,  a  teaspoonful  hourly  or 
25-50  c.c.  (1-2  ounces)  per  rectum.  Formerly,  Hquor  ferri  sesqui- 
chlorati  (P.  G.)  in  h  per  cent,  mucilaginous  solution  (one  teaspoonful 
hourly,  or  fluid  extract  of  ergot,  0.25  c.c.  (n^  4)  in  two  ounces  of  water, 
one  teaspoonful  every  hour  were  recommended.  In  place  of  the  last- 
named  drug,  ergotin  may  be  used  subcutaneously.  Because  of  the 
rapid  fall  of  the  body  temperature,  the  children  should  be  wrapped  in 
absorbent  cotton  and  flannels  and  kept  warm  by  means  of  thermo- 
phores and  hot  bottles  (not  applied  to  the  abdomen,  but  best  to  the  lower 
extremities).  Local  applications  of  cold  are  not  to  be  used.  The  admin- 
istration of  small  quantities  of  tea  or  the  subcutaneous  injection  of 
physiological  salt  solution  is  recommended  to  support  the  heart,  when 
large  quantities  of  blood  have  been  lost. 

In  cases  of  spurious  melajna,  the  source  of  the  bleeding,  if  acces- 
sible, is  to  receive  appropriate  treatment. 


PREMATURITY  AND  CONGENITAL  DEBILITY 

BY 

Tin.  O.  ROMMEL,  of  Mun:ch 

tr.vnslated  by 
Dr.  a.  S.  MASCHKE,  Cleveland,  O. 


Prematurity  and  congenital  debility  are  often  looked  upon  as  syn- 
onymous terms.  However,  it  is  apparent  from  the  very  meaning  of  the 
words  that  this  idea  is  false;  the  word  prematurity  (partus  pra?maturus) 
means  only,  birth  before  term,  whereas  the  e.xpression  debiUty  (dcbihtas 
vitse)  is  qualitative,  in  the  sense  that  the  respective  cliild  is  deficient, 
compared  vdth  a  healthy  newborn  infant. 

The  confusion  of  terms  is,  however,  apparently  justified  by  the 
fact  that  premature  children  are  often  debilitated;  wliich  occurs  when 
one  and  the  same  poison  {e.g.,  parental  syphiUs)  is  responsible  for  both 
the  premature  interruption  of  pregnancy  and  the  damage  to  the  infan- 
tile organism.  These  constitute  the  diseased,  debihtated,  premature 
infants,  on  the  one  side  of  whom  we  can  place  the  healthy  premature 
infants,  and  on  the  other  side,  the  debilitated  full-term  infants.  Tarnier 
speaks  truly  when  he  says  "not  all  premature  children  are  weaklings 
and  not  all  weaklings  are  premature." 

It  will,  in  the  future,  in  accordance  with  the  increase  of  our  knowl- 
edge of  these  topics,  be  necessary  to  treat  these  conditions  separately. 
At  present,  in  view  of  the  existing  hterature  and  in  accordance  with  the 
practical  purpose  of  this  manual,  this  hardly  seems  feasible  and  more- 
over would  lead  to  rather  needless  repetition.  UtiUty,  therefore,  impels 
us  to  adhere,  in  the  follo\ving,  to  a  consideration  of  these  themes — pre- 
maturity and  congenital  debihty — in  common;  their  principal  points  of 
variance  will  be  succinctly  emphasized  in  the  separate  subdivisions. 

Although  the  term,  prematurity,  needs  no  further  explanation, 
the  expression,  debihty,  requires  exacter  definition.  Billard  charac- 
terized it  as  a  condition  lying  between  health  and  disease.  Very  httle 
is  known  of  the  anatomical  changes  or  metabohc  anomalies  which  are 
at  the  bottom- of  tliis  status  " debilUe  congenitale."  It  is  characterized 
by  a  quantitatively  and  quahtatively  deficient  vital  energy  and  a  low- 
ered resistance  to  all  infections. 

Occurrence. — As  shown  by  the  statistics  of  l^Ting-in  institutions, 
premature  children  constitute  a  forinidable  percentage  of  the  total 
number  of  births.     The  percentage  varies  widely  \\'ith  the  locahty  and 

II— 6  81 


82  THE    DISEASES   OF   CHILDREN 

the  country;  thus  the  number  of  cliildren  under  2500  Gm.  (5i  lbs.)  in 
weight  and  less  than  45  cm.  in  length  were: 

In  Moscow  (orphan  asylum)  5  per  cent.  (Miller). 

In  Munich  (maternity)  13.3  per  cent,  {von  Winckel). 

In  Halle  (maternity)  2.5  per  cent.  (Fehling). 

In  Paris  (Clinique  Tarnier)  10.7  per  cent.  (Btulin). 

In  Paris  (Maternite  and  Chnique   Bandelocque)   15.4  per  cent.    (Pinard). 

It  is  stated  that  the  percentage  of  premature  births  increases  in 
the  spring  months,  sinks  during  the  autumn  and  is  larger  in  winter  than 
in  summer. 

Etiology. — Many  causes  exist  for  the  occurrence  of  prematurity 
and  they  differ  wdely  in  their  importance. 

Thus  external  influences,  such  as  severe  physical  exhaustion,  moun- 
tain cUmbing,  the  lifting  of  heavy  objects,  traumata  of  various  sorts, 
premature  rupture  of  the  foetal  membranes,  etc.,  can  furnish  the  impetus 
for  premature  labor.  Twin  pregnancy  is  also  a  frequent  etiological  factor. 
In  3380  plural  births.  Miller  observed  no  less  than  2040  premature 
children,  that  is  60  per  cent,  with  a  weight  less  than  2500  Gm.  {oh  lbs.) 
and  a  body  length  under  45  cm.;  Bachimont  found  in  super-impreg- 
nated women,  who  were  unable  to  take  adequate  rest,  that  the  dura- 
tion of  pregnancy  was  shortened,  on  the  average,  by  22  days  and  the 
weight  of  the  children  brought  down  to  1900-1935  Gm.  It  is  at  present 
impossible  to  say  to  what  extent  faulty  nutrition  and  physical  excesses 
as  well  as  psychic  alterations  in  the  mother,  act  in  producing  partus 
prjematurus.  Maternal  diseases  play  an  important  role  in  the  etiology 
of  both  prematurity  and  congenital  debility.  Foremost  in  tliis  connec- 
tion is  syphiUs — and  this  ex  patre  as  well — which,  by  extension  to  the 
foetus  affects  its  development  and  leads  to  partus  immaturus  or  praema- 
turus.  Other  maternal  diseases  wliich  lead  to  the  premature  expulsion 
of  the  foetvis  are  overshadowed  in  importance  by  the  last-mentioned 
cause;  these  other  diseases  are:  nephritis,  heart  disease  and  tubercu- 
losis. Of  the  acute  infections,  scarlet  fever  is  rightly  the  most  dreaded. 
Prematurity  is  said  to  occur  in  two-thirds  of  the  cases  of  pneumonia 
and  to  increase  in  probability  with  advancing  pregnancy.  Its  occurrence 
with  influenza  depends  upon  the  severity  of  the  attack.  The  influence 
of  malaria  has  been  variously  estimated,  although  A\ith  it,  the  spleen 
of  the  newborn  infant  can  be  enlarged.  According  to  Voigt,  prema- 
turity occurs  with  variola  in  about  one-half  the  cases  befalling  mothers 
vaccinated  in  girlhood.  Measles,  tj'phoid,  bubonic  plague,  and  Asiatic 
cholera  can  hke^^^se  give  the  impetus  for  a  premature  expulsion  of  the 
foetus;  and  gonorrhoea  more  frequently  than  was  formerly  assumed. 
Endometritis  leads  to  abortion  more  often  than  prematurity.  Besides 
acute  and  chronic  alcohohsm,  wliich  are  particularly  prone  to  cause 
still-birth,  there  are  various  other  poisons  which  produce  intoxication  of 


PREMATURITY   AND    CONGENITAL   DEBILITY 


83 


both  mother  and  child  and  can  give  rise  to  miscarriage  or  prematurity; 
these  are  especially  phosphorus,  arsenic,  mercury,  and  lead.  Typical 
signs  of  lead  poisoning  haA'e  been  observed  in  a  premature  cliild. 

Physiology  and  Pathology. — The  weight  of  the  premature  child 
depends,  on  the  one  hand,  on  its  age,  on  the  other,  on  the  cause  of  the 
premature  labor.  The  extremes  vary  between  750  Gm.  (1§  lbs.)  and  3000 
Gm.  (64  lbs.).  According  to  Ahlfeld  and  Hecker,  the  averages  of  body 
weight  and  length  in  round  numbers,  are  as  follows: 


27  weeks 1140  Gm.  2%  lbs. 

29  weeks 1575  Gm.  3      lbs. 

31  weeks 1975  Gm.  4K  lbs. 

33  weeks 2100  Gm.  i%  lbs. 

35  weeks |  2750  Gm.  6      lbs. 

37  weeks I  2875  Gm.  6%  lbs. 


Weight. 


Length. 


.W.3  cm.  14      in. 

:19.6  cm.  \a\:,  in. 

42.7  cm.  16VI  in. 

4:1.9  cm.  17     in. 

47..3  cm.  18J^  in. 

48.3  cm.  19      in. 


These  figures  have  only  an  approximate  worth,  as  can  be  seen  from 
the  folloAnng  statistics  of  French  writers,  shoeing  the  widely  varying 
weights  of  premature  infants. 


27  weeks. 

29  weeks. 

31  weeks. 

33  weeks. 

35  weeks. 

Author. 

1146  Gm.   2i<Jlbs. 

140SGm.   2Jilbs. 

995  Gm.   2}6lbs. 

IMOGm.    S     lbs. 
1700  Gm.    ::'.Ubs. 
1676  Gm.   :'.'.^lbs. 

livSlGm.   354  lbs. 
1900  Gm.   3%  lbs. 
1964  Gm.   Sjjlbs. 

2213  Gm.   4%  lbs. 
21.50  Gm.    4'.8lbs. 
2182  Gm.   4>8lbs. 

2400  Gm.   5     lbs. 
2766Gm."53^ii)s'. 

Francois 

Potel 

Hahn 

In  case  the  average  weight  is  less  than  that  of  a  healthy  foetus  of 
the  corresponding  age,  it  is  justifiable  to  conclude  that  a  greater  or  less 
number  of  these  observed  children  were  debilitated  or  at  least  ill. 

In  every  case  one  must  bear  in  mind  that  the  weight  especially, 
(even  more  so  than  the  body  length)  is  indiAidually  extremely  vari- 
able; thus  we  have  a  type  of  children,  usually  from  parents  of  slight 
stature,  who  are  small  in  structure,  and  although  they  weigh  onl}'  2000 
Gm.  (44  lbs.)  they  are  in  no  way  debihtated  and  show  no  signs  of  prema- 
turity. According  to  Pinard,  we  find  placentte  of  very  small  weight 
with  such  cliildren. 

It  is  other\\'ise  ■RTth  hereditary  syphiHs  in  which  strikingly  large 
placentae  are  found  with  the  tiniest  weakUngs.  The  children  of  albu- 
minuric and  nephritic  mothers  are  also  small  and  puny  and  have  been 
termed  "spiders"  by  Pinard.  The  statistics  of  Berthod  demonstrate 
how  inadequate  an  aid  the  weight  of  the  premature  is  for  determining 
its  age;  these  statistics  include  48  cliildren  born  at  eight  months  or 
more,  weigliing  less  than  2000  Gm.  (44  lbs.)  and  among  these  seven  full- 
term  infants;  also  fifty-two  infants  born  at  less  than  eight  months 
whose  weight  was  more  than  2000  Gm.  (44  lbs.),  and  among  these  two  born 
at  six  months.    The  body  length  may  be  taken  as  a  reUable  criterion. 

Body  Temperature  and  Energy  Balance. — Uncommon  sensi- 
tiveness  to   low   temperatures  is   pecuhar   to   premature   children  and 


84  THE   DISEASES   OF   CHILDREN 

weaklings.  The  temperature  of  a  healthy  newborn  infant  falls  a  few- 
tenths  of  a  degree  centigrade  after  birth,  but  ordinarily  soon  returns  to 
normal.  Tliis  is  not  the  case  with  the  premature,  and  especially  not, 
with  the  debilitated  premature  child.  In  these  cases  the  temperature 
falls  steadily  to  32°  C.  (90°  F.)  and  even  lower,  unless  the  cliild  is  placed 
in  an  especially  favorable  environment.  On  an  average  the  less  the 
weight  of  the  cliild  and  the  more  debihtated  it  is,  the  lower  its  tempera- 
ture. The  reasons  for  this  lasting  hypothermia — whether  a  primarily 
deficient  oxidation  (Bonniot)  or  an  inactivity  of  the  thermo-regulative 
apparatus  (nervous  influence)  as  certain  French  authors  are  iucUned  to 
assume — remain  unsettled. 

After  the  initial  drop,  the  temperature  curve  takes  the  form  of  a 
parabola.  The  longer  the  child  is  without  artificial  heat,  the  flatter  the 
curve.  A  retarded  rise,  when  in  the  incubator,  is  an  unfavorable  sign, 
just  so  an  abrupt  rise  after  a  previously  stationary,  subnormal  tempera- 
ture. Also,  premature  cliildren  characteristically  show  an  instabihty 
of  body  temperature  in  contrast  to  the  shght  nocturnal  variations  of 
the  healthy  young  nurshng.  A  deficient  thermal  regulation,  which 
according  to  Babak  is  characteristic  of  all  newborn  infants,  is  found  in 
an  exaggerated  degree  in  premature,  debihtated  cliildren.  Much  energy 
is  lost  through  increased  loss  of  heat  through  the  skin,  on  account  of 
the  relatively  greater  body  surface  of  the  premature  child  wliich  also 
lacks  adipose  tissue.  Added  to  this,  these  cliildren  lack,  as  do  all 
newborn  infants,  the  physical  thermo-regulative  capacity;  whereas  the 
chemical  thermal  regulation  (increase  in  oxidation  processes),  wliich, 
in  these  cliildren  and  especially  the  debihtated  ones,  is,  without  tliis, 
at  a  very  low  point,  becomes  taxed  beyond  its  capacity.  This  leads  to 
changes  in  the  alkahnity  of  the  blood  (lowered  to  one-quarter  the  nor- 
mal) and  to  the  deposition  of  waste  products  and  toxins  in  the  body 
(Charrin,  Guillemonat,  Levaditi).  Pfaundler,  examining  debihtated 
premature  cliildren,  found  the  actual  reaction  of  the  blood  to  be  acid. 
(The  concentration  of  the  OH-ions  smaller  than  that  of  the  H-ions.) 

A  more  favorable  balance  of  energy,  showing  a  certain  excess  of 
energy  necessary  for  increase  in  size  and  weight,  is  only  possible  by 
lowering  the  loss  of  heat  by  wrapping  the  child  in  a  non-conductor  of 
heat,  by  hot-water  bottles  or  by  raising  the  temperature  of  the  en- 
\dronment — the  use  of  the  incubator.  When  one  considers  the  diffi- 
culty of  nourisliing  premature  cliildren,  and  their  deficient  assimilation, 
it  becomes  plain  that  the  balance  of  energy  very  readily  becomes  nega- 
tive; that  is,  the  child  loses  ground.  More  exact  knowledge  concerning 
the  dynamic  exchange  in  premature  or  debihtated  children  is  not  at  hand. 

Behavior  of  Weight  and  Further  Development. — The  initial 
loss  of  weight  is,  according  to  the  investigations  of  Delestre  and  the 
writer,  less  than  in  full-term  children.     It  depends  on  the  quantity  of 


PREMATURITY   AND    CONGENITAL   DEBILITY  85 

meconium,  on  the  feeding,  especially  the  ingestion  of  water,  and  on 
external  circumstances.  The  separation  of  the  cord  plays  no  part  in 
this  connection.  The  gain  in  weight  is,  at  first,  despite  sufficient  feed- 
ing, slower  than  in  the  normal  child,  however  it  sometimes  attains  the 
average,  after  reaching  the  normal  time  of  the  termination  of  labor. 
The  further  development  of  the  muscular  and  osseous  systems,  and  of 
the  teeth,  and,  finally,  of  the  psychic  functions,  is,  in  healthy  premature 
infants,  backwards  about  the  extent  of  time  that  the  children  are  born 
prematurely.  Czerny  and  Keller  certainly  speak  truly  when  they  assert 
that  these  cliildren  in  later  infancy  are  hardly  to  be  recognized  from 
full-term  infants.  Debilitated  and  sick  premature  children,  on  the  con- 
trary, often  remain  for  years  behind  their  contemporaries. 

Special  Pathology  of  the  Organs. — The  organs  of  unhealthy 
premature  cluldrcn  are  in  a  state  of  insufficient  development;  func- 
tionally they  are  usually  backward  and  quantitatively  deficient.  In  de- 
bilitated children  we  may  have,  in  addition  to  the  signs  of  prematurity, 
other  physiological  changes,  causing  ciuahtative  de^^ation  from  the 
normal;  these  may  or  may  not,  depending  upon  the  organ  affected,  l)e 
a  menace  to  the  life  and  impair  the  future  development  of  the  affected 
child. 

The  Nervous  System. — In  contrast  to  the  healthy  newborn  infant 
the  sleep  of  the  premature  cliild  is  even  and  deep  and  after  lasting  some 
time  does  not  become  hghter.  Besides  the  somnolence,  the  indolence  of 
the  child's  movements  and  the  absence  of  all  reactions  are  noticeable. 
The  intelligence  and  psycliic  function  develop  more  slowly — a  fact  wliich 
possibly  depends  on  the  insufficient  development  of  certain  centres. 

Spasmophiha,  as  it  has  been  described  by  certain  writers,  is  not 
characteristic  of  premature  children.  Spasms,  wliich  occur  immediately 
after  birth,  are  not  uncommonly  the  results  of  birth  injuries.  Intra- 
cranial ha:'morrhages  and  cerebral  diseases  are  especially  apt  to  occur  in 
small,  first-born,  premature  children,  according  to  WaUich.  Premature 
infants,  in  fact,  have  a  predisposition  to  nervous  affections.  Thus  pre- 
maturity plays  a  prominent  role  in  the  etiology  of  Little's  disease. 
Among  100  such  cases  Audebert  found  82  in  premature  children. 
According  to  French  writers,  concUtions  of  psycliic  depression  and 
paralysis  in  childhood  are  found  mth  relatively  great  frequency  in 
premature  children.  Just  how  far  hereditary  S3'pliilis  is  involved  in 
these  affections  of  the  nervous  sj'stem  remains  unsettled;  however, it  is 
certain  that  nian}^  an  instance  of  cerebral  atrophy  is  only  the  sequelum 
of  a  foetal  encephaUtis. 

Circulatory  Apparatus. — The  heart  is  comparatively  strong;  how- 
ever cardiac  weakness  sometimes  causes  cyanosis  and  oedema  (C.  Hahii). 

In  a  case  of  prematurity  under  my  observation,  I  was  able  to 
satisfy  myself  that  atelectases,  by  increasing  the  resistance  in  the  lesser 


86  THE    DISP:ASES   OF   CHILDREN 

circulation,  can  exert  a  retarding  influence  on  and  even  entirely  interfere 
with  the  physiological  closure  of  the  foramen  ovale  and  ductus  Botalh. 

The  fragile,  brittle  condition  of  the  arterial  wall  in  these  cliildren 
may  have  a  connection  with  sypliiUs  (mesarteritis  syphiHtica,  Heubner). 

The  blood  of  premature  children  shows  a  deficient  coagulability; 
there  is  a  great  tendency  to  hiemorrhages,  especially  epistaxis,  and 
also  mela?na  (sepsis?).  In  young  premature  children  one  finds  nu- 
cleated red  cells.  Delestre  found  that  these  forms  disappear  when  the 
temperature  of  the  cliild  rises;  they  reappear,  however,  with  any  ill- 
ness of  the  child.  Leucocytosis  was  only  present  in  sUght  degree.  The 
decrease  in  the  alkahnity  of  the  blood  has  already  been  mentioned. 
According  to  Adriance,  the  percentage  of  haemoglobin  is  excessively 
high,  however,  it  gives  way  soon  after  birth  to  an  increased  destruction 
of  cells;  tliis  fact  probably  has  some  connection  with  the  occurrence  of 
icterus,  the  non-syphiUtic  forms  as  well,  in  these  children. 

The  lymphatic  apparatus  shows  in  contrast  to  the  full-term  child, 
a  noteworth)'  anatomical  completeness;  however,  0T\ang  to  the  slow- 
ness of  its  circulation,  it  probably  more  easily  permits  of  the  escape  of 
toxins  and  bacteria. 

The  Respiratory  Apparatus. — The  breatliing  of  httle  premature 
children  is  shallow  and  irregular.  The  voice  is  monotonous  and  feeble. 
On  auscultation  one  can  barely  hear  the  vesicular  respiratory  mur- 
mur. The  percussion  note  is  flattened  toward  the  bases.  Not  uncom- 
monly, on  account  of  the  diminished  respiratory  excursion,  the  lower 
parts  of  the  lungs  remains  in  a  condition  of  atelectasis;  the  air  does  not 
reach  the  alveoh,  but  only  the  finer  bronchi;  tliis  explains  the  predis- 
position to  pneumonia  that  exists  in  these  children.  Accompanjing 
this  we  have,  as  especially  important  in  the  pathology  of  the  premature 
child,  attacks  of  cyanosis  and  asphyxia  which  occur  soon  after  birth, 
on  account  of  pulmonary  atelectasis. 

These  attacks  often  occur  without  warning  in  well-developed 
cliildren,  the  face  and  hands  becoming  cyanotic.  Respiratory  pauses, 
one  or  more  minutes  in  dvu-ation,  recur  so  that  the  breathing  resembles 
the  Cheyne-Stokes  type.  Convulsions  are  also  observed.  The  pulse  is 
greatly  slowed-down  to  40  beats  per  minute  or  less. 

The  cause  of  these  attacks  is  not  clearly  established.  Finkelstein 
beheves  them  to  be  the  consequence  of  a  subtly  developing  carbonic- 
acid  intoxication,  which  is  brought  about  by  insufficient  breatlung  or 
also  by  meteorismus  and  the  consequent  encroachment  upon  the  intra- 
thoracic capacity. 

Budin  considers  them  to  be  the  consequence  of  chronic  under- 
feeding and  sees  them  disappear  with  the  institution  of  forced  feechng. 
From  several  personal  observations,  I  have  gained  the  impression  that 
they  are  cerebral  in  origin,   but  cannot  substantiate  this  behef  with 


PREMATURITY    AND    CONGENITAL    DEBILITY  87 

autopsy  findings.  Occasionally  tiie  attacks  seem  to  be  elicited  by  the 
feeding  of  the  child. 

Billard  and,  after  him,  other  writers  have  described  a  condition 
in  the  premature  child,  vnth  which  respiration  is  entirely  wanting. 
The  heart's  action  is  weak  and  slowed.  The  blood  flows  from  the  right 
heart  through  the  ductus  Botalh  into  the  aortic  system  without  ever 
passing  through  the  lungs.  In  this  manner  a  "vita  minima"  can  be 
maintained  for  a  few  days;  such  observations,  however,  belong  to  the 
rarities  and  are  mthout   practical  importance. 

The  Digestive  Tract. — Marfan  considers  the  digestive  tract  to 
be  the  most  vulnerable  "organ-system"  of  the  premature  child;  still 
the  difficulties  can  usually  be  overcome  through  a  properly  instituted 
feeding.  Small  premature  cMldren  are  incapable  of  suckUng  and  even 
larger  ones  are  thereby  easily  exhausted.  Swallowing  is  slowed.  The 
digestive  fluids  (ferments)  are  quantitatively  and  quahtativelj'  deficient 
as  compared  with  the  normal,  although  exact  researches  concerning 
this  are  not  at  hand.  French  authors  write  of  functional  disturbances 
of  the  hver  in  weakUngs.  The  meconium,  which  is  usually  scant,  is 
delayed  in  its  passage;  Hkewise  one  often  encounters,  in  the  first  weeks, 
a  constipation  or  rather  pseudo-constipation  wMch  is  the  result  of 
diminished  intestinal  activity  or  underfeeding. 

Finkelstein  has  repeatedly  observed  an  atrophy  of  the  stomach 
occurring  in  the  later  months  and  this  justifies  his  warning  against  the 
overfeeding  of  premature  infants. 

Skin  and  Adnexa. — The  skin  is  more  or  less  intensely  reddened 
and  covered  with  lanugo  hairs;  the  redness  persists  somewhat  longer 
than  with  full-term  cliildren;  the  external  ears  he,  as  foldless  skin- 
tabs,  close  against  the  skull;  the  nails  are  either  absent  or  else  fall 
short  of  the  end  of  the  phalanges;  the  insertion  of  the  umbilical  cord 
lies  deeper  than  normal,  and  the  epidermal  desquamation  is  slowed. 
Owing  to  the  absence  of  the  adipose  layer  in  the  subcutaneous  cellular 
tissue,  the  skin  lies  loose  and  movable  over  the  underlying  parts.  Ero- 
sions occur  easily  after  the  shghtest  injuries;  these  losses  of  substance 
occur  especiall}'  over  the  malleoli  and  the  heels.  Besides  sclerema  and 
scleroedema,  to  wiiich  these  children  are  very  prone,  there  occurs  a  form 
of  icthyosis  described  by  French  authors,  concerning  which,  however, 
nothing  definite  is  known. 

The  Kidneys. — The  frequent  occurrence  of  uric  acid  infarctions 
is  expUcable  through  the  deficient  oxidation  and  the  insufficient  circu- 
lation and  respiration;  the  ingestion  of  too  small  a  quantit}^  of  water 
may  also  have  sometliing  to  do  with  this.  Uric  acid  infarction  can 
give  ri.se  to  a  suppression  of  urine  lasting  for  one  or  more  days,  which 
in  turn  occasionally  leads  to  cohcky  and  convulsive  states  (encephalo- 
pathie  ur^mique,  Parrot). 


88  THE   DISEASES   OF   CHILDREN 

Baumel  cites  a  case  ending  in  recovery,  in  which  a  premature  child 
urinated  through  the  umbilicus  (urachus).  According  to  Charrin  the 
(juantity  of  urine  is  low  and  the  acidity  and  toxicity  are  raised. 

The  proportion  of  ammonium  N  to  total  N  is  less  than  the  normal; 
^  on  the  contrary  is  increased,  which  points  to  an  increase  in  the 
decomposition  processes.  According  to  Nobecourt  and  Lemaire,  a 
lowering  of  the  freezing  point  (A)  of  the  urine  of  premature  infants  is 
likewise  present. 

Hernia',  especially  umbilical  hcrniir,  occur  with  relative  frequency 
in  premature  children. 

The  bacterial  infections,  which  can  occur  intraruterine,  intra- 
partum, or  extra-uterine,  play  a  weighty  and  characteristic  j)art  in  the 
pathology  of  the  premature  child. 

The  extra-uterine  infections,  wluch  usually  lead  rapidly  to  death, 
can  take  place  through  the  skin,  the  mucous  membranes,  the  respiratory 
tract,  the  eyes,  mouth,  intestinal  tract,  and  navel.  According  to  Fischl 
and  Delestre,  with  whom  I  herein  agree,  the  lungs  afford,  especially  in 
hospital  patients,  the  most  frequent  point  of  entry  for  liacterial  inva- 
sion; in  this  connection,  a  subnormal  body  temperature  furnishes  a  pre- 
disposing element.  These  children  usually  die  within  a  few  days,  of  a 
hannorrhagic  form  of  pneumonia,  occurring  under  the  chnical  picture 
of  asphyxia,  usually  running  an  afebrile  course.  Delestre  found  coryza 
occurring  only  in  premature  children  with  a  normal  temperature. 

Henry  describes  a  rhinitis,  running  a  chronic  course  in  premature 
children,  which,  contrary  to  the  associated  assumption,  is  not  specific. 

Diagnosis. — The  diagnosis  of  prematurity  is  made,  in  extreme 
cases,  from  the  typical  general  appearance.  Besides  the  small  size, 
the  characteristic  signs  of  unripeness  strike  the  eye. 

The  most  important  clews,  besides  the  duration  of  the  pregnancy, 
are  furnished  by  the  weight  and  measurement  of  the  child. 

Fewer  external  characteristics  come  into  consideration  for  the  diag- 
nosis of  debiUty. 

A  low  body  weight— under  2500  Gm.  (5i  lbs.)  (Czerny  and  Keller) 
can,  as  these  authors  themselves  declare,  hardly  be  considered  as  a 
sharp  boundary.  It  is  rather  the  general  behavior  of  the  child,  a  dimin- 
ished resistance  to  external  harmful  influences  (temperature,  infection, 
artificial  feeding,  etc.),  wMch  inform  us,  often  not  until  weeks  after 
the  birth,  that  we  are  deahng  with  a  debiUtated  child. 

Prognosis. — The  prognosis  of  a  premature  child  depends  first  and 
foremost  on  its  absolute  age,  which  is  its  degree  of  ripeness.  A  child 
which  is  born  before  the  twenty-seventh  to  twenty-eighth  week  of  preg- 
nancy has  only  a  small  chance  of  remaining  alive. 

The  weight  takes  second  place  as  an  indicator,  and  therefore  a 
small   older    child  is  better  off  than  a  large    younger  one.     Notwith- 


PREMATURITY   AND    CONGENITAL   DEBILITY  Si) 

standing  this,  statistics  rightly  show  a  decrease  in  the  mortahty  with 
increasing  natal  weight.  It  is  palpably  clear  that  a  child  weighing 
1800  Gm.  (4  lbs.),  other  things  being  equal,  has  better  chances  than  an- 
other weighing  1200  Gin.  {2h  lbs.) ;  then  again,  the  heavier  child  can  have 
a  worse  prognosis,  provided  it  be  not  only  premature  but  also  at  the 
same  time  debihtated. 

Hereditary  influences:  parental  diseases,  especially  maternal  (syphi- 
lis, tuberculosis),  are  very  important  for  determining  the  capacity  for 
hfe  of  the  Httle  neonate.  When  the  premature  birth  is  artificially  in- 
duced, because  of  some  mechanical  reason  (contracted  maternal  pelvis) 
the  prognosis  is  naturally  better. 

According  to  Budin,  children  having  a  rectal  temperature  of  32° 
C.  (90°  F.)  and  less,  die  almost  uniformly.  The  behavior  of  the  child 
immediately  after  delivery  is  very  important  prognostically.  Children 
that  cry  lustily,  move  in  a  Uvely  fashion,  drink  well,  or  even  suck  of 
their  own  accord  on  the  proffered  breast,  have  much  better  prospects 
than  those  children,  apathetic,  hungry,  and  cold,  with  temperatures  of 
hardly  32°  C.  (90°  F.)  that  are  not  given  medical  attention  until  days 
after  birth. 

Although  the  prognosis  at  first  is  dubious,  it  later  becomes  better 
in  proportion  as  the  care  and  nutrition  are  entirely  satisfactory,  and 
comphcations  do  not  occur. 

Mortality. — Potel's  statistics  show  the  influence  of  the  absolute 
age  in  the  mortahty. 

Of    .56  children  of  6J  foetal  mont  h.s 45  =  SO. 4  per  cent,  died 

Of  131  children  of  7    foetal  mont hs 76  =  58. 1  per  cent,  died 

Of   53  children  of  7i  foetal  months 17  --  30.1  per  cent,  died 

Of  110  children  of  8    foetal  month,s 39  -^  35.5  per  cent,  died 

Credc  established  a  mortahty  of  83  per  cent,  for  children  weigh- 
ing 1000-1500  Gm.  (2^-3  lbs.);  a  mortahty  of  36  per  cent,  for  those 
weighing  1500-2000  Gm.  (3-4i  lbs.);  and  11  per  cent,  for  those  weigh- 
ing 2000-2500  Gm.  (4i-5i  lbs.). 

In  these  statistics,  however,  one  must  remember  tiiat-the  healthy 
and  debihtated  premature  children  are  not  separated.  Francois  sepa- 
rated these  two  classes  and  arrived  at  the  fact  that  of  81  premature 
children  of  diseased  parents  (syphihs,  tuberculosis,  albuminuria)  30  to 
37  per  cent,  died;  whereas  of  386  approximatel_y  health}''  premature 
children,  only  48  (12.5  per  cent.)  died.  Just  how  much  can  be  done,  even 
in  institutions,  in  the  way  of  decreasing  the  mortahty  rate  of  premature 
children,  is  shown  by  the  pubhcations  of  Hutinel  and  Delestre,  who 
were  enabled  to  reduce  the  death  rate  from  66  per  cent,  to  36  per  cent, 
and  later  to  14  per  cent.,  "thanks  to  the  almost  motherly  care,  the  iso- 
lation of  the  .sick,  the  use  of  the  conveuse,  the  mother's  milk  and  a  sort 
of  air-cure." 


90 


THE   DISEASES   OF   CHILDREN 


Fig.  10. 


According  to  Groth  of  Munich,  the  mortahty  from  congenital 
debiUty  during  the  first  month  of  hfe,  is  lowest  in  August  and  highest 
in  the  ^\'inte^  months. 

Aside  from  the  first  two  weeks,  the  mortahty  of  premature  children 
is  hardly  liigher  than  that  of  healthy  full-term  ones. 

According  to  Budin,  15  per  cent,  of  the  premature  cliildren  died 
after  discharge  from  his  institution  and  17.4  per  cent,  of  the  full-term 
ones,  after  discharge. 

Pathological  Anatomy. — Post-mortem  examination  of  young  pre- 
mature cliildren  distinctly  discloses  tlieir  unripeness,  besides  an  ana?mia 
of  the  organs.  The  organs  correspond  to  the  fcetal  development.  The 
lungs  are  very  ana?mic  and  show  either  partial  or  total  atelectasis. 
The  brain,  of  colloid  consistency,  permits  only  of  inaccurate  differentia- 
tion into  white  and  gray  substance,  the  gyri  are 
imperfectly  developed,  the  pyramidal  tracts  in 
the  spinal  cord  are  still  undeveloped.  The  kid- 
neys plainly  show  renculin  formation.  The 
thymus,  thyroid,  and  adrenal  are  noticeably 
large.  The  foetal  channels  (ductus  Botalh, 
ductus  Arantii,  and  foramen  ovale)  are  still  open, 
showing  insufficient  involution.  The  epiphyseal 
osteogenctic  centres  are  very  small  and  undevel- 
oped. In  sypliilitic  premature  cliildren,  besides 
other  specific  manifestations,  one  finds  the  osteo- 
chondritis of  Wegner  (see  chapter  on  sypliilis). 
Prophylaxis. — The  prophylaxis  is  a  twofold 
one.  In  so  far  as  it  concerns  the  occurrence  of 
a  premature  birth,  it  does  not  come  within  the 
range  of  tliis  book  but  rather  belongs  to  the  pro- 
\ince  of  the  obstetrician  or  sypliilographer.  The 
prophylaxis  of  the  premature  child  consists  in 
keeping  away  all  external  harmful  influences; 
it  is  curative,  in   the  furnishing  of  skilled  care, 


100 


80 


70 


60 


so 


40 


30 


20 


10 


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ms^^- 

Mortality  statistics  for 
premature  infaats. 


in  its  po.sitive  phase 
warmth  and  natural  food. 

Treatment. — The  treatment  of  premature  and  debihtated  children 
consists  (1)  in  the  furnisliing  of  warmth;  (2)  in  careful  feeding;  and  (3) 
in  the  avoidance  of  harmful  influences,  particularly  bacterial.  Other 
measures,  especially  medicinal,  are  of  much  less  importance. 

1.  Furnishing  warmth  to  the  body,  immediately  after  birth,  is, 
according  to  the  unanimous  opinion  of  all  authors  on  this  subject,  the 
first  and  most  important  measure  in  the  treatment  of  the  premature 
child.  A  bath  of  37°  C.  (98.6°  F.)  increased  to  40°  C.  (104°  F.),  and 
the  careful  tran.sportation  of  the  infant  into  a  pre\-iou.sly  prepared  warm 
room,  most  surely  secure  this.    A  bath  of  the  above-given  temperature 


PREMATURITY   AND    CONGENITAL   DEBILITY 


91 


lasting  fifteen  to  twenty  minutes  is  also  the  best  measure  to  quickly 
bring  the  fallen  temperature  back  to  normal,  in  cliildren  that  only  come 
under  treatment  later.  Eross  rightly  states  that  not  all  premature 
children  require  artificial  warming.     However,  the  small  and  the  debili- 


FlG.    11. 

OB-        Jan.    Feb.    Mar.    Apr.    May    June    July    .Vug.    Sep.    Oct.    Nov.    Dec. 


230 


iZO 


tlO 


100 


190 


ISO 


170 


Dusea  ;es 
1st  771  imth 


Conge 
0-1  d. 


ttiiid 


intay 


Compare  extreme  height  of  the  mortahty  in  summer,  due  to  gastro-intestiual 

diseases,  with  the  zero  of  the  deaths  from  debihty  in  August. 

(Course  of  the  mortality  in  the  first  month  of  life,  in  Munich.      Separated  according  to  cause  of  death.) 

tated  ones  do  require  it  in  one  or  another  form.  The  supply  of  heat 
should  be  accurately  measured  for  it  has  distinct  indications  and  con- 
traindications. In  case  the  three  hourly  rectal  temperatures  show  a 
subnormal  temperature,  artificial  warming  is  demanded.     Whereas  in 


92  THE    DISEASES   OF   CHILDREN 

private  practice  one  usually  must  get  along  with  crude  appliances,  in 
maternity  hospitals  and  clinics  for  nurslings  one  usually  finds  appa- 
ratus for  furnishing  warmth  (incubators),  wliich  with  advancing  im- 
provement and  decreasing  cost  will  be  in  the  near  future  more  widely 
used  in  private  houses. 

The  principal  requisites  of  an  incubator  are  (1)  simphcity  and 
surety  in  working,  (2)  cleanliness,  (3)  artificial  Ught,  ventilation  and 
moistened  air*,  (4)  accurate  regulation  of  the  temperature,  that  is, 
dosage  of  the  supply  of  heat.  We  must  admit  unfortunateh'  that  des- 
pite the  greatest  care  there  is  hardly  an  existing  model  which  fulfils 
all  these  demands. 

Although  the  problem  of  the  therapy  of  the  premature  is  in  no  way 
fully  solved  by  furnisliing  a  good  incubator,  and  even  though  its  use  be 
confined  to  those  cases  where  a  strict  indication  is  at  hand,  still  the 
results  obtained  from  its  employment  are  so  convincing  (compare 
Berthod,  Gagey  and  others),  that  the  greatest  possible  perfection  in 
such  apparatus  seems  well  worth  while. 

The  opponents  of  the  incubator  may  content  themselves  with  the 
dictum  of  Delestre,  who  says,  "Tant  vaut  le  mileau,  tant  vaut  la  con- 
veuse. " 

I  will  omit  the  historical  description  of  the  various  models. 

An  apparatus  which  is  a  modification  of  the  French  model  devised 
by  Finkelstein  (Fig.  12),  and  in  use  in  the  City  Children's  Asylum  of 
BerUn,  appears  to  be  both  practical  and  simple.  It  is  made  of  galvan- 
ized steel  tin,  ^\^th  rounded  edges.  The  box,  which  rests  on  a  stand,  is 
divided  into  two  parts;  an  upper  compartment,  partly  of  glass,  which 
is  the  receptacle  for  the  child;  and  a  lower  compartment,  the  hot  water 
receptacle,  closed  by  means  of  a  sUding  panel.  Cold  air  enters  through 
openings  located  on  the  side  of  the  lower  part,  is  there  heated,  and 
then  passes  through  the  upper  compartment,  leaving  it  by  means  of 
the  air  vent.  The  necessity  of  regularly  changing  the  hot  water  con- 
tainers, makes  punctual  attendance  imperative. 

The  author's  incubator  (Fig.  13)  with  its  latest  improvements, 
can,  similarly,  be  erected  very  cheaply  in  either  house  or  chnic.  It  is 
divided  into  a  compartment  for  the  child;  the  heat  and  moisture  gen- 
erator; a  water  container  and  heat  box.  The  accompanying  diagram 
makes  a  detailed  description  unnecessary.  The  cubic  contents  of  the 
incubator  is  0.09  cbm.,  antl  4.97  cbm.  of  air  pass  through  it  in  the  course 
of  an  hour,  consequently  the  air  changes  fifty  times  hourly. 

Besides  Schlossmann's  electric  heated  incubator,  the  one  of  Huti- 
nel  also  deserves  mention.  In  view  of  the  danger  of  infection  which  has 
often  been  attributed  to  the  conveuse,  the  latter  is  made  of  varnished 


*  The  quantity  of  air  should  be  1.5  cm.  an  Iiour  for  each  Itilogram  in  weight  of  the  cliiltl.  The  huniidity 
should  be  about  60  per  cent. 


PREMATURITY   AND    CONGENITAL    DEBILITY 


93 


Fig.  12. 


fayence,  shaped  like  a  bath  tub.  The  rest  of  the  apparatus  is  very  simi- 
lar to  the  Finkelstein  one;  the  heating  appliance  being  changed  every 
two  to  three  hours. 

Several  institutions  (Children's  Hospital,  Gratz,  St.  Anna  Children's 
Hospital,  Vienna,  and  elsewhere)  have  incubator  rooms  (see  Fig.  14). 
These  are  far  superior  to  the  simple  incubators  and  according  to  the 
judgment  of  experienced  chnicians  are  well  adapted  for  use  in  insti- 
tutions; they  can  even 
at  times  be  improvised 
in  private  practice,  if 
one  has  an  abundant 
supply  of  heaters  at 
command. 

The  room  in  wiiich 
a  premature  cliild  is 
kept,  mth  or  without 
a  conveuse,  should  be 
of  an  even  tempera- 
ture, from  about  22°-25° 
C.  (71.o°-77°  F.),  and 
should  conform  to  the 
inexpensive  require- 
ments of  modern  hy- 
giene, at  least  as  far  as 
cleanliness,  ventilation, 
and  sunshine  are  con- 
cerned. The  air  in  the 
room  should  be  kept 
sufficiently  moist.  The 
temperature  in  the  in- 
cubator must  be  kept 
from  26°-28°  C.  (79°- 
82°  F.),  at  the  start; 
only  very  small,  prema- 
ture cliildren  require 
a  higher  temperature.  It  hardly  seems  practical  to  keep  the  tempera- 
ture of  the  incubator  constant,  at  25°  C.  (77°  F.)  as  recommended  by 
Pinard. 

If  the  body  temperature  is  not  raised  by  the  above-mentioned 
temperature  of  the  incubator,  the  humidity  of  the  air  must  be  increased 
by  placing  hot,  moist  towels  in  the  incubator — "conveuse  humlde" 
recommended  by  von  Bonnaire  and  Gagey,  by  means  of  wliich  these 
authors  have  attained  surprising  results. 

With   the   shghtest   signs   of   overheating    (restlessness,    sweating, 


Finkelstein's  incubator. 


94 


THE   DISEASES   OF   CHILDREN 


increased  respiration,  cyanosis)  the  body  temperature  must  be  reduced, 
if  necessary  by  remo^'ing  the  child  from  the  incubator. 

Hutinel  and  Delestre  make  use  of  the  incubator  for  as  short  a  time 
as  possible,  and  after  maintaining  an  even  temperature  from  one  to  two 
days,  recommend  the  removal  of  the  child  from  the  incubator.  Like- 
\vise,  every  acute  infection  furnishes  a  contraindication  to  the  use  of 
the  incubator.  One  can  use  as  a  substitute  for  the  incubator  treatment, 
heated  tubs,  hot  water  bottles,  thermophores  in  the  form  of  heat-pillows 
j-j^^  j3  (liighly  praised  by  Heubner),  as  well 

as  the  electro-thermostat  lately  put 
on  the  market. 

In  using  the  above-given  meth- 
ods, the  breatliing  air  does  not  be- 
come heated,  while,  on  the  other 
hand,  an  over-heating  of  the  cliild, 
by  insufficient  radiation  of  the  heat, 
can  readily  take  place.  Neverthe- 
less, the  simplest  way  is  by  placing 
two  to  four  hot  water  bottles  in  the 
bed  wliich  must  be  refilled  every  two 
hours.  The  rectal  temperature  must 
be  taken  regularly  so  that  any 
stasis  of  heat,  which  may  cause 
serious  trouble,  can  be  detected. 

The  U-shaped  hot  water  bot- 
tle, which  appears  in  the  accom- 
panying illustration  Fig.  15,  has 
proved  itself  very  useful  because 
frequent  refilling  is  unnecessary. 

2.  Feeding  and  its  technique  are 
particularly  important  in  the  ther- 
apy  of   premature   and    debilitated 
i,,,n,iuri.- u^ubaioi.  children.    Above  all  tilings  mother's 

milk  is  desirable.  It  must  be  considered  a  grave  error,  if,  through  any 
negligence,  the  opportunity  to  obtain  mother's  milk  is  lightly  passed 
by.  This  apphes  in  a  still  greater  degree  for  debiUtated  cliildren  (espe- 
cially with  hereditary  syphilis),  whose  prognosis  actually  depends  on 
their  being  nursed  at  the  breast  (Heubner). 

The  greatest  difficulty  is  the  inabiUty  of  the  premature  child  to 
nurse  and  in  private  residences  it  is  especially  a  very  difficult  task  to 
accustom  the  child  to  the  breast;  in  priniipara;  with  poorly  secreting 
breasts  or  retracted  nipples,  this  is  next  to  impossible.  Only  the  great- 
est patience  in  expressing  and  pumping  the  milk  by  means  of  a  strong 
breast-pump,  to   avoid  caking,  or  still  better,  the  comcomitant   nurs- 


PREMATURITY   AND   CONGENITAL   DEBILITY 


!)5 


^"s-Q-^'if »-  P--  '^^  S"  =  ' 

"  »  a  a  °  =  i^  O  S  si  S  5"  S-  a  -" 


96 


THE   DISEASES   OF   CHILDREN 


ing  of  a  stronger,  older  infant,  will  bring  the  breast  into  operation. 
With  tliis  purpose  in  view,  it  is  advisable  to  place  both  mother  and 
child  in  a  nursUng's  home,  or  some  similar  institution.  Wet-nurses 
advantageously  continue  nursing  their  own  cliildren  along  with  their 
charge,  thus  keeping  the  milk  supply  up  to  the  highest  point.  In  select- 
ing a  wet-nurse  for  a  premature  cluld,  the  greatest  care  should  be  exer- 
cised to  detect  the  possible  existence  of  a  latent  syphihs.  Occasionally 
a  skilfully  directed  "allaitement  mixte"  accomphshes  the  desired  result. 
The  child,  thus  strengthened,  attacks  the  breast  more  vigorously 
and  thereby  increases  the  secretion  of  the  mother's  milk.  It  is  of  great 
importance  to  know  the  daily  amount  of  nourishment  necessary,  and 

thereby   avoid   both   overfeeding 

Fig.  l.^.  1       !»        T  T-»       1 

and  underieeding.  Both  are 
harmful.  Nevertheless  the  great- 
est danger  in  practice  seems  to  be 
the  underfeeding  of  small  prema- 
ture cliildren.  They  seem  to  re- 
quire a  relatively  greater  amount 
of  food  at  this  time  than  in  the 
later  nursing  period.  The  volume 
of  food  each  day  to  be  deter- 
mined by  either  the  scale  or  grad- 
uate, for  the  first  ten  days  of 
life  equals  V=n-|-10  (in  c.c;  n  = 
number  of  days)  per  100  Gm.  For 
example,  a  child  weighing  1500 
Gm.  requires  on  tlie  fifth  day  of 
fife  about  5-|-10=15  per  cent,  of 
the  l)ody  weight,  which  equals  225  Gm.  of  mother's  milk.  Later  a 
premature  child  should  drink  about  one-fifth  of  its  body  weight  per 
day,  and  at  full  term  one-sixth. 

The  amount  of  energy,  in  this  volume  of  food,  required  by  a  flour- 
ishing infant  varies  from  130-120-110  calories  per  kilo  of  body  weight, 
diminisliing  with  the  increasing  weight  and  age  of  the  child. 

Technique  of  Feeding. — As  it  is  not  possible  to  feed  large  quanti- 
ties at  a  single  meal  to  small  premature  infants  (often  only  10-20  Gm.; 
3iiss-5v)  it  becomes  necessary  to  nourish  them  every  two  hours  or  even 
at  shorter  intervals,  i.e.,  ten  to  twenty  times  in  twenty-four  hours. 
Czerny  and  Keller  only  give  six,  sometimes  five  meals  in  twenty-four 
hours.  In  feeding  according  to  tliis  schedule,  it  seems  impossible  to 
avoid  underfeeding. 

In  very  small  premature  infants  the  nourishment  must  be  intro- 
duced (gavagp)  by  means  of  a  Nelaton  catheter,  or  allowed  to  slowly 
trickle  into  the  mouth  or  the  nose  by  means  of  a  pointed  spoon.     The 


U-shaped  liot  water  bottle  for  premature  infants. 


PREMATURITY   AND    CONGENITAL    DEBIIJTY 


97 


glass  flasks  depicted  in  the  accompanying  illustration  Fig.  16  (Undine's) 
have  proved  very  useful  in  oral  and  nasal  feeding. 

Before  feeding,  small  premature  infants  must  often  be  stimulated 
by  means  of  slapping,  pinching,  etc.  A  short  bath  of  37°-39°  C.  f9S.6°- 
102°  F.),  or  a  cool  sponging  is  often  useful.  Many  cliildren  immediately 
eject  their  nourishment  and  it  requires  great  patience  and  skill  on  the 
part  of  the  nurse  to  make  them  retain  it. 

The  artificial  feeding  of  premature  and  debihtated  children  will 
always  come  into  account  only  as  a  last  resort ;  the  result  will  always 
be  uncertain  and  it  is  cUfficult  to  recommend  any  one  method.  The 
formula  must  be  controlled  by  the  general  condition  and  by  the  stools, 
and  must  resemble  mother's  milk  as  nearly  as  possible  in  amount  and 
caloric  value.   •  Overfeeding  with  its  fig.  le. 

harmful  results  must  be  strictly 
avoided  in  the  artificial  feeding  of' 
premature  infants.  Self-prepared 
whey — milk  mixtures  of  2:1  and 
1:1;  also  milk  diluted  with  an  equal 
quantity  of  calf's  broth,  are  indi- 
cated. There  is  a  division  of  opin- 
ion as  to  the  amount  of  cream  neces- 
sary for  premature  infants.  I  have 
no  experience  with  the  butter-milk 
carbohydrate  mixtures,  lately  recom- 
mended by  Finkelstein.  Budin  and 
Michel  praise  a  mixture  in  which 
the  albumin    has    been   peptonized 

by  the  action  of  a  fresh  extract  of  calf's  pancreas.  Although  there 
are  many  references  in  the  Htcrature  recommending  peptonized  milk 
for  the  feeding  of  premature  infants,  I  cannot  conscientiously  recom- 
mend the  use  of  the  factory  preparations  of  milk. 

The  prevention  of  infection,  of  every  sort,  is  most  successfully 
accomplished  by  skilful  attendance.  The  cord  should  be  carefully 
dressed  with  hydrophiUc  gauze,  moistened  with  1-2000  bichloride  solu- 
tion. Cleansing  the  mouth  should  be  omitted  as  it  is  useless  and 
moreover  harmful  since  the  oral  epithehum  is  very  easily  injured.  The 
bath  water  should  be  boiled.  Sponge  baths  \Aith  warm  water  and 
very  fatty  soap  are  preferable  to  tub  baths  at  first.  The  new  dusting 
powders,  or  talcum  with  the  addition  of  boric  acid  1:2,  should  be  used. 
Ointments  as  a  rule  are  very  poorly  tolerated.  The  customary  cloth- 
ing is  used  and  should  always  be  previously  warmed;  only  very  small 
premature  infants  should  be  wrapped  in  cotton.  All  chiUing  and  the 
too  early  airing  of  the  premature  child  are  to  be  avoided. 

II— 7 


used  for  nasal  or  mouth  feedine. 


98  THE   DISEASES   OF   CHILDREN 

Of  the  remaining  tlierapeutic  measures  for  premature  and  debili- 
tated cliildren,  we  will  only  mention  the  following: 

The  use  of  oxygen  (Bonnaire  and  Geneay  recommend  its  use  as  a 
prophylactic  as  well  as  in  cyanosis,  asphyxia,  infections  and  vomiting). 

Blood-letting.  (Delestre  recommends  repeated  lileedings  of  2-3  c.c. 
in  children  who  do  not  gain  sufficiently;  also  a  single  bleeding  of  a  larger 
amount,  18-20  cm.). 

Injections  of  artificial  serum  (20-30  c.c.)  are  also  recommended 
by  Delestre  and  other  French  authors.  Budin  regards  massage  with 
hot  oil,  repeated  2-3  times  daily  as  very  useful. 

Complications  are  to  be  treated  accordingly. 


ASPHYXIA  AND  ATELECTASIS 

BY 

Dr.  O.  ROMMEL,  of  Munich 

TRANSLATED    BY 

Dr.  a.  S.  iL\SCHKE,  Clevel.\.nd,  O. 


AsPHYXi.\  is  a  disease  of  the  newborn,  with  which  tlie  interchange 
of  gases  of  the  blood — absorption  of  oxygen  and  ehmination  of  carbon 
dioxide — is  either  suspended  or  more  or  less  diminished.  Through  the 
diminished  ventilation  of  the  blood,  a  pathological  oxygen-deficit  en- 
sues and  an  overloading  with  carbon  dioxide,  a  condition  of  asphyxia, 
which  seriously  threatens  life.  If  the  respiration  is  utterly  wanting  one 
speaks  of  apparent  death  ("Scheintod")- 

We  differentiate  two  forms  of  asphyxia: 

I.  The  congenital  form,  usually  beginning  sub  partum,  and  wlaich 
occurs  as  (a)  asphyxia  cyanotica  I  degree,  and  (6)  asphyxia  palhda, 
II  degree  (Runge). 

II.  The  acquired  form,  which  occurs  after  birth  and  which  on  ac- 
count of  its  usual  cUnical  and  pathological  findings  has  also  been  termed 
atelectasis. 

Both  forms  are  to  be  .sharply  differentiated  in  respect  to  etiology, 
occurrence  and  course;  tliis  however  does  not  prevent  both  forms  from 
occasionally  occurring  in  the  same  child  or  going  over  into  one  another. 

I.    CONGENITAL    ASPHYXIA 

The  discussion  of  this  form  in  this  place  -nill  be  short,  since  it  really 
belongs  to  the  province  of  the  obstetrician. 

Etiology. — The  premature  excitation  of  the  respiratory  centre, 
through  wliich  futile  inspirations  are  elicted  ante  partum,  can  occur  in 
many  waj's: 

I.  Causes  on  the  part  of  the  infant: 

(a)  Compression  or  t'^isting  of  the  umbilical  cord. 
(6)  Premature  detachment  of  the  placenta, 
(c)  Abnormal  cerebral  pressure  in  the  foetus. 

II.  Causes  on  the  part  of  the  mother: 

(o)  Insufficient   maternal   circulation   and   arterialization    (espe- 
cially with  heart  and  lung  affections). 

(6)   Lowering   of   the    maternal    blood    pressure    on    account    of 
hsemorrhages,  agony,  death  of  the  mother,  labor. 

(c)  Anomalous  labor  pains,  e.g.,  tetanus  uteri. 

99 


100 


THE   DISEASES   OF   CHILDREN 


With  the  increase  in  the  carbon  dioxide  contents  of  the  infantile 
blood,  the  irritabihty  of  the  respiratory  centre  in  the  medulla  decreases 
and  severe  paralysis  of  the  respiratory  function  of  the  newborn  can 
arise,  through  which  the  lungs  can  remain,  even  after  birth,  in  the  fa^tal 
state  of  atelectasis. 

Symptomatology  and  Course. — Tliis  asphyxia,  arising  intra  utero 
from  the  above-mentioned  causes,  begins  almost  always  shortly  before 
birth,  often  develops  rapidly  and  ma}'  become  a  serious  menace  to  the 
life  of  the  cliild. 

Of  the  symptoms  indicating  intra-uterine  asphyxia,  besides  the 
passing  of  meconium,  the  most  rehable  is  the  weakening  of  the  foetal 
heart  sounds.      This   symptom,  due   to  irritation   of   the  vagus,   gives 

place,   in   severer    stages    of 

-    ^^^..  .,.■-...  ..,.'.';.    .        ..'••-.. .'TCw        the  asphyxia,  to  a  consider- 

-  \  able    increase   in   the   heart 

sounds  (vagus  paralysis)  and 
demands  the  immediate  end- 
ing of  the  labor.  The  asphyc- 
tic newborn  is  cyanotic, 
varying  in  color  from  a  blu- 
ish-red to  a  deep  blue  (as- 
phyxia cyanotica  first  degree 
of  Runge).  It  Hes  motion- 
less \\"ith  a  swollen  face  and 
closed  eyelids,  its  Uttle  legs 
sMghtly  flexed.  The  breath- 
ing is  superficial  and  infre- 
quent and  is  accompanied 
often  by  ratthng  and  hic- 
coughing. The  heart's  action 
is  strong  and  usually  infre- 
quent. The  muscular  tone 
and  also  the  reflex  excitability  are  retained.  Reflex  choking  movements 
are  ehcited  on  introducing  the  finger  into  the  pharynx  in  order  to  aid  in 
the  removal  of  mucus.  By  irritating  the  skin,  deeper  inspirations  are 
eUcited  wliich  however  usually  soon  diminish  in  intensity. 

With  the  second  degree  (asphyxia  palhda)  the  skin  is  pale,  the  Ups 
alone  are  bluish,  the  muscular  tone  is  wanting,  all  the  extremities  hang 
loosely  relaxed,  reflex  irritabihty  is  lost  and  the  heart's  action  is  fre- 
quent and  weak.  Respiration  is  entirely  stopped,  at  the  most  one 
observes  here  and  there  a  jerky,  almost  convulsive,  movement  of  the 
whole  cliild.  The  upper  air-passages  are  usually  totally  occluded  and 
thus  the  entrance  of  air  is  hindered  by  mucus  and  amniotic  fluid, 
aspirated  in  consequence  of  premature  respirator)'^  movement. 


■  N;^?^:v!:-^^- 


st-::^:- 


Pulmonary  atelectasis  in  the  newborn.    The  alveoli  CD 

are  collectively  collapsed.  The  interspaces  (2)  represent  the 
alveolar  ducts.  (3)  Transition  of  a  bronchiole  to  an  alveolar 
duct.     (4)    Cross-section  of  bronchus. 


ASPHYXIA   AND   ATELECTASIS  101 

Gradualh"  the  heart's  action  ceases  and  the  body  temperature 
sinks  and  thus  these  cliildren  usually  die;  others  occasionally  drag  on 
for  a  few  days,  but  only  to  die.  Where  the  treatment  avails,  respira- 
tory movement  starts,  the  eyes  are  opened  and  the  child  moves  with 
increasing  liveliness.  The  skin  becomes  rosy  and  feels  warm,  the  pulse 
strong  anfl  regular,  mucus  is  expectorated  (vomited)  for  days. 

Pathological  Anatomy. — The  signs  of  death  from  asphyxia  are 
most  cATdent :  tliin,  watery  blood;  the  right  ventricle  and  the  large 
vessels  distended,  as  also  the  veins  leading  from  the  brain  and  liver; 
the  liver  is  dark  blue  in  color;  petecliial  and  larger  haemorrhages  under 
the  pleura,  pericardium,  peritoneal  covering  of  the  liver,  the  pia  and 
other  organs;  also  htemorrhagic-serous  effusions  into  the  pleural,  peri- 
cardial and  peritoneal  ca^^ties. 

Besides  tliis  are  the  signs  of  attempts  at  respiration  before  de- 
livery; congestive  hypersemia  of  the  lungs;  ecchymoses  under  the 
pleura  and  pericardium.  The  respiratory  passage  (lar3'nx  and  bronchi) 
is  filled  mth  mucus,  amniotic  fluid  or  meconium  and  one  can  often  trace 
these  masses  through  to  the  finer  bronchi. 

In  cliildren  who  are  born  asphyctic  but  have  hved,  one  finds,  be- 
sides the  more  or  less  extensive  atelectases,  also  air-containing  areas; 
the  latter  much  lighter  in  color  and  raised  above  the  atelectatic  areas; 
the  atelectases  dark  and  leathery.  Amniotic  fluid  and  meconium  are 
not  rarely  found  in  the  stomach  since  swallowing  movements  may  occur 
with  the  attempts  at  ante-natal  respiration. 

Diagnosis. — The  diagnosis  of  asphy.xia  is  made  from  the  clinical 
picture  and  doubt  can  also  arise  as  to  whether  in  a  given  case  one  has 
to  do  with  a  combination  with  cerebral  compression  or  not;  a  not  un- 
common condition  in  first-born  premature  infants. 

According  to  Runge  the  diagnosis  of  an  asphyxia  complicating 
cerebral  compression  is  justifiable  when,  with  an  asphyxia  of  the  first 
degree,  the  breathing  continues  irregular  and  more  infrequent  and  the 
pulse  rate  diminishes  in  spite  of  the  institution  of  energetic  cutaneous 
stimuH.  In  case  one  suspects  an  acute  anaemia  search  should  be  made  for 
the  cause  of  such  an  anii^rnia,  e.g.,  velamentous  insertion  of  the  cord. 

Prognosis. — Unless  skilful  treatment  is  employed  the  prognosis 
is  grave.  Usually  the  asphyctic  cliildren  of  the  first  degree  go  over 
into  the  second  degree,  whereas  the  latter  die.  With  suitable  treatment 
the  milder  cases  usually  recover  and  even  the  severe  cases  are  not 
entirely  hopeless  if  peristent  treatment  be  kept  up. 

The  opinion  advanced  by  Schultze  and  Jacobi  concerning -the  ap- 
pearance of  a  later  idiocy,  after  severe  and  protracted  asphyxia,  and  also 
the  reports  of  Little  and  Jlitchell  wliich  refer  to  the  connection  between 
birth  trauma  and  nervous  and  psychic  lesions,  are  not  taken  into  con- 
sideration prognostically,  in  indi\'idual  cases. 


102  THE   DISEASES   OF   CHILDREN 

Prophylaxis. — Tliis  falls  within  the  province  of  obstetrics. 
Therapy. — The  treatment  of  asphyxia  neonatorum  consists  in  the 
use  of  the  following  measures: 

1.  In  the  Clearing  out  of  the  Air  Passages. — If  mucus  or  amniotic 
fluid  penetrate  into  the  air  passages,  one  must  aspirate  through  a  Ne- 
laton  catheter,  steadily  sucking  wliile  advancing  the  catheter.  The 
method  of  Ahlfeldt  and  Pinard,  which  consists  in  suspending  the  child 
by  its  legs,  for  a  quarter  of  a  minute,  thereby  causing  the  congestive 
hyperemia  to  act  in  the  greatest  degree  on  the  respiratory  centre,  also 
seems  practical.  At  the  same  time  the  mucus  should  be  aspirated. 
Prochownick  recommends  rhythmic  compression  of  the  thorax  while 
the  child  is  in  this  suspended  position. 

2.  The  Use  of  Active  Cutaneous  Irritants. — Alternating  hot  and  cold 
douches  are  most  effective.  The  child  is  immersed  to  its  neck  alternately  in 
warm  water  of  40°  C.  (104°  F.)  and  cold  water  of  20°  C.  (68°  F.).  The  treat- 
ment must  always  start  and  end  with  warm  water  immersions;  besides 
this,  the  usual  cutaneous  irritations  by  slapping  the  gluteal  region. 

3.  In  every  severe  case  of  asphyxia  (II  degree),  uninterrupted, 
prolonged  artificial  respiration  must  be  employed.  The  writer  considers 
Schultze's  swinging  method  to  be  the  most  effective  of  all.  In  regard  to 
the  well-understood  technique  of  this  method*,  we  need  only  note  that 
after  six  to  eight  swinging  movements,  the  child  should  always  be  im- 
mersed in  warm  water  of  38°  C.  (100°  F.),  in  which  one  must  employ 
thorough  friction,  and  rapid  rhythmical  compression  of  the  heart  in 
order  to  raise  the  cardiac  action  to  120-140  a  minute.  The  suspicion, 
recently  raised,  that  Schultze's  method  might  give  rise  to  rupture  of 
internal  organs  with  consequent  hremorrhages,  lacks  sufficient  proof. 
One  should  never  despair  of  resuscitating  the  infant  as  long  as  any  sign 
of  cardiac  action  be  present  and  restorative  measures  must  be  kept  up 
until  the  child  cries  long  and  lustily  (Schultze). 

Sylvester's  method  of  artificial  respiration  also  deserves  mention; 
it  consists  in  the  strenuous  abduction  and  adduction  of  the  arms  and 
shoulders,  thus  increasing  or  decreasing  the  intra-thoracic  capacity. 

Laborde's  method  by  traction  on  the  tongue  is  difficult  of  execution 
in  the  newborn,  on  account  of  the  smallness  of  the  part  to  be  manipulated. 

The  method  of  Pernice,  consisting  in  the  use  of  faradic  electricity, 
has  been  abandoned,  since  by  it  only  inspirations  can  be  elicited.  Con- 
trariwise, however,  the  results  from  the  u.se  of  oxygen  have  been  rather 
gratifying. 

II.    ACQUIRED    ASPHYXI.\ 

This  is  also  called  atelectasis  pulmonum  and  is  encountered  in 
premature  and  debiUtated  children.     AVe  differentiate  according  to  the 


*See  text  books  on  Obstetrics. 


ASPHYXIA   AND   ATELECTASIS  103 

onset  an  early  and  a  late  form  of  asphyxia.     (Concerning  the  latter  see 
also  the  chapter  on  prematurity  and  debility). 

The  etiology  of  this  form  of  asphyxia  is  rather  complex. 

I.  General  debiUty  with  which  all  the  functions  are  quantitatively 
and  qualitatively  impaired  and  a  high  degree  of  somnolence  exists; 
with  this,  through  a  Ungering  carbonic  acid  intoxication,  paralysis  of 
the  respiratory  centre  results  (Finkelstein). 

II.  Cerebral  diseases;  especially  injuries  in  the  region  of  the  me- 
dulla, from  birth-traumata  (haemorrhages?);  also  congenital  hydro- 
cephalus. 

III.  Pulmonary  affections  (aplasia  of  the  lungs,  white  pneu- 
monia); also  congenital  struma  or  hyperplasia  of  the  thymus  which 
leads  to  compression  of  the  trachea. 

IV.  A  yielding  thoracic  wall  and  costal  cartilages,  as  well  as  a  poorly 
developed  respiratory  musculature  in  premature  cliildren. 

V.  Acute  fatty  degeneration  of  the  newborn  (Buhl's  disease)  wliich 
in  the  absence  of  hiemorrhages  can  be  masked  through  the  symptom  of 
asphyxia. 

VI.  Underfeeding  (Budin)  as  well  as  overfeeding  (Henry)  are 
held  responsible  as  etiological  factors  in  cases  of  asphyxia  in  premature 
children. 

The  course  of  asphyxia  occurring  soon  after  birth,  especially  in 
premature  and  debilitated  children,  is  usually  as  follows:  the  children 
usually  slumber  apathetically,  without  demanding  nourishment,  and  are 
noticeably  quiet.  The  face  is  at  times  slightly  puffed  and  shght  oedema 
occurs  on  the  extremities,  especially  on  the  backs  of  hands  and  feet. 
The  temperature  is  subnormal.  The  breatliing,  tolerable  at  first,  be- 
comes more  superficial  and  irregular;  now  quicker  and  now  interrupted 
by  longer  pauses.  Ausculation,  after  having  spanked  the  child  a  few- 
times,  reveals  crepitant  rales  usually  over  the  bases  (atelectatic  crepi- 
tations). In  some  children  one  observes  locahzed  lateral  retractions, 
also  at  times  in  the  middle  of  the  sternum.  Now  and  then  severer  at- 
tacks of  cyanosis  intervene  without  warning.  With  a  falling  tempera- 
ture and  marked  loss  of  weight,  the  children  die  usually  within  a  few 
days  and  often  even  within  a  few  hours.  Now  and  then  the  asphyxia 
occurs,  especially  in  premature  children,  as  late  as  a  few  weeks  after 
birth  and  is  then  usually  a  very  bad  sign. 

The  pathological  anatomical  findings  are  often  totalh-  negative 
except  for  a  more  or  less  extensive  pulmonary  atelectasis. 

The  diagnosis  is  furnished  by  the  pulmonary  findings,  the  impair- 
ment of  respiration,  the  increasing  stupor  and  the  poor  appetite. 

The  prognosis  depends,  first,  on  the  cause  underhing  the  asphyxia. 
It  depends  further  on  the  treatment  instituted.  Should  any  improve- 
ment of  the  condition  be  secured  by  means  of  the  therapeutic  measures. 


104  THE   DISEASES   OF   CHILDREN 

one  usually  wins  the  battle.  This,  however,  does  not  hold  good  for  the 
cases  of  asphyxia  occurring  later  in  premature  children,  which  usually 
terminate  fatally. 

The  treatment  of  acquired  asphyxia  consists  chiefly  in  the  use  of 
hydrotherapeutic  measures.  The  alternating  hot  and  cold  baths  recom- 
mended, under  2,  for  congenital  asphyxia,  often  prove  valuable  when 
frequently  repeated.  Heubner  recommends  baths  at  35°  C.  (95°  F.) 
of  only  short  duration,  combined  with  pouring  cold  water  10-12°  C. 
(50-53i°  F.)  over  the  chest,  back  and  head  four  to  six  times,  using  one 
pint  each  time  and  repeating  regularly  every  two  hours.  Besides  this, 
warmth  and  breast-feeding.  In  these  cases  oxygen  inhalations  are  espe- 
cially recommended.  The  other  therapeutic  measures  recommended 
for  congenital  asphyxia  may  also  be  symptomatically  employed. 


SCLERCEDEMA  AND  SCLEREMA 

BY 

Dr.  O.  ROMMEL,  of  Mujjich 

translated   by 
Dr.  a.  S.  M.\SCHKE,  Clevel.a.xd,  O. 


Both  these  diseases,  between  which  formerly  no  differentiation 
was  attempted,  have  now  obtained  an  assured  place  in  the  pathologj-  of 
the  newborn  and  the  young  nursUng. 

In  the  older  literature  a  considerable  confusion  dominated  the 
subject  of  these  affections.  The  confusion  with  the  scleroderma  of 
adults*  contributed  in  no  way  to  the  clearing  of  the  situation.  How- 
ever, since  the  writings  of  Denis  and  Billard,  most  authors  have  sought 
to  differentiate  two  forms,  a  serous  and  an  adipose  hardening  of  the  skin. 
The  "arbeit"  of  Clementowsky,  wliich  was  grounded  on  accurate  cUni- 
cal  and  anatomical  investigations,  marks  a  noticeable  advance  in  the 
knowledge  of  these  diseases.  And,  although  according  to  Luithlen  no 
entire  clarit}^  exists  to-day  concerning  these  topics  and  discussion  even 
exists  in  the  text  books,  still  this  does  not  accord  with  the  view  of  the 
writers  on  pediatrics  (Parrot,  Baginskj%  Henoch,  Widerhofer,  Comby, 
Soltmann). 

SCLERCEDEMA;    SCLEREMA  CEDEMATOSUM 

Symptomatology. — The  disease  usually  begins  with  vague  pro-: 
dromal  signs,  such  as  lessening  of  the  appetite,  slight  restlessness  and 
crying;  and  at  the  same  time  the  breathing  becomes  shallow  and  irreg- 
ular and  the  heart's  action  weaker.  After  a  few  hours  the  oedema  is 
seen  on  the  back  of  the  feet,  on  the  cheeks  and  also  on  the  mons  veneris. 
The  oedema  spreads  upward,  leaving  the  chest  free,  and  is  most  extensive 
on  the  lower  extremities.  The  hands  and  arms  are  also  attacked  but 
rarel}^  the  ej'eUds  and  the  rest  of  the  face.  The  penis  and  scrotum  are 
in  hke  manner  swollen.  The  skin  over  the  affected  parts  is  tense  and 
usually  cj'anotic  in  premature  children:  but  in  cliildren  born  at  term 
or  when  the  affection  occurs  somewhat  later,  after  the  physiological 
e.xfohation  has  terminated,  the  skin  is  pale,  waxy  and  at  times  mottled. 

An  increase  in  volume  is  apparent;  the  consistency  in  less  severe 
cases  is  that  of  butter  (Heubner),  but  in  advanced  cases  the  skin  is  hard 
and  stiff  and  distinctly  gives  the  sensation  of  coldness  to  the  palpating 
finger.    The  child  hes  still  and  apathetic,  the  temperature  in  mild  cases. 


*  Scleroderma,  the  scleroderma  of  adult.^;,  which  occasionally  occurs  in  children  and  even  in  young 
nurslings  (Cruse,  Neuman,  et  al.)  has  nothing  in  common  with  sclerema  or  scleroderma  of  the  newborn. 

105 


106 


THE   DISEASES   OF   CHILDREN 


Fig.  18. 


35-34°  C.  (95°-93.2"  F.),  sinks  in  severe  cases  to  32°  C.(89.3°  F.)  and  lower. 
The  excretion  of  urine  is  scant  and  its  amount  is  of  some  prognostic 
value.  Albumin  is  usually  not  present.  The  body  weight  does  not 
always  diminish,  as  is  usually  stated,  but  on  the  contrary  may  even 
increase. 

In  the  severer  cases  the  children   die   with  gradual  weakening  of 
respiration  and  the  heart's  action,  and  with  increasing  stupor.     Death 
takes  place  usually  after  four  to  five  days,  in  protracted  cases  after  one 
to  two  weeks,  although  cases  of  lesser  severity  frequently  end  in  recovery. 
Actual  compUcations  are  rare.    Pneumonias   occurring  simultane- 
ously, diseases  of  the  navel,  pem- 
phigus and  sepsis  are  to  be    con- 
sidered as  independent   affections. 
Occurrence. — Scleredema  oc- 
curs only   in    the    newborn.      Sel- 
dom congenital,  it  begins,  as  a  rule, 
on  the  second  to  fourth  day  of  life, 
rarely  later,  up  to  the  second  week. 
Premature  and  debilitated  children, 
twins    and    hereditary   syphilitics 
are    especially  affected.     It  is  also 
rather  often  observed  with  congeni- 
tal heart  disease  and  nephritis.    Less 
severe   forms   arc    very   frequently 
encountered  in  premature  children. 
In    winter    and    in     localities 
where    the   cUmate  is   cold,  many 
more   cases   come   under  observa- 
tion.    The  disease  is  encountered 
more    frequently  in  hospitals  and 
dispensaries,    that   is,  it    is    more 
common   among   the    poorer  class 
of  people  than  in  private  practice. 
Pathogenesis,  Nature. — This  disease,   concerning  the  etiology  of 
w'liich  much  uncertainty  exists,  is  dependent,  for  its  origin,  on  several 
factors. 

The  peculiar  anatomical  relations  in  the  newborn,  and  especially 
in  the  premature  or  debihtated  newborn,  furnish  a  suitable  basis  for 
its  occurrence. 

On  the  one  hand  muscular  and  circulatory  weakness,  on  the  other  a 
lowering  of  the  oxidation  processes  and  of  respiration,  are  involved  in 
the  causation  of  scleroedema.  The  influence  of  cold  on  the  infantile 
organism  becomes  the  exciting  factor. 

The  nervous  theory  (Liberal!,  Ballantyne,  G.  Somma)  and  also  the 


# 


Scleroedema  in  the  newborn.  Thinning  of  the 
epidermis  and  flattening  of  the  papillse,  extensive 
softening  and  thickening  of  the  coriura  with  widen- 
ing of  the  lymph-spaces  and  lymphatic  vessels. 


SCLEROEDEMA    AND    SCLEREMA  107 

theory  of  an  infectious  origin,  are  more  hypothetical  and  have  received 
no  general  recognition.  Luithlen  unquahfiedly  denies  the  existence  of 
sclercedema  as  an  entity;  he  classes  it  with  the  other  oedemas  of  the 
newborn,  mth  which  it  sliares  a  common  etiological  basis,  differing  only 
by  the  superaddition  of  the  elements  of  cold. 

Pathological  Anatomy. — Except  for  an  occasional  degeneration 
of  the  heart  muscle  (Demme)  the  usual  findings  are  a  venous  congestion, 
especially  in  the  distribution  of  the  vena  cava;  and  then  congestion  of 
the  lungs,  atelectatic  areas  and  small  ha?morrhages  in  the  lungs  and 
pleurs.  The  cedema  itself  is  not  necessarily  confined  to  the  skin  and  the 
subcutaneous  tissues  but  may  on  the  contrary  spread  to  the  deeper 
IjTng  muscles. 

Reference  is  made  to  the  illustration  for  the  liistological  findings. 

The  diagnosis  is  easily  made  in  pronounced  cases.  The  pitting  of 
the  skin,  on  pressure  -nith  the  examining  finger,  serves  to  differentiate 
the  rarely-occurring  sclerema,  wluch  feels  much  harder  and  vnth.  which 
the  penis  and  scrotum  are  uninvolved. 

Acute  erysipelas  is  differentiated  by  its  color,  localization  and  the 
fever  usually  accompanying  it. 

The  prognosis  is  favorable  in  mild  cases,  but  becomes  more  dubious 
the  more  extensive  the  involvement;  and  also  when  other  complications 
(atelectasis,  pneumonia,  heart  disease)  are  present. 

Prophylaxis. ^Tliis  consists  in  the  prevention  of  any  immoderate 
chilling,  especially  -nith  premature  and  debihtated  cliildren,  and  the 
instituting  of  breast-feeding. 

Treatment. — The  treatment  consists  primarily  in  the  furnisliing  of 
artificial  heat  (conveuse).     (See  chapter  on  prematurity  and  debility.) 

The  stimulation  of  respiration  by  means  of  ox3'gen  inhalation,  com- 
bined with  artificial  respiration,  is  recommended.  Hot  baths,  38-42° 
C.  (100.2-107.3°  F.),  with  massage  and  passive  motion  in  the  bath  or 
after  it  (Soltmann);  inunctions  with  glycerine  to  wliich  10  per  cent,  of 
iodide  of  ammonium  has  been  added  are  recommended  bv  Badaloni; 
diuretics  and  digalen  1-1-2  drops  internally.  Hot  sweetened  coffee 
(50-100  Gm.),  possibly  per  rectum. 

Where  there  is  difficulty  in  swallowing,  gavage  and  nutrient  ene- 
mata.     Breast-feeding  must  be  employed  if  possible. 

SCLEREMA 

Clinical  Description. — The  onset  of  sclerema  is  similar  to  that  of 
sclercedema  in  that  it  affects  the  lower  extremities,  especialh'  the  calves, 
in  a  symmetrical  arrangement.  On  careful  palpation  even  in  the  early 
stages  of  the  disease,  a  doughy  sensation  can  be  felt  in  the  deeper  laj-ers 
of  the  skin.  This  soon  extends  over  the  thighs,  trunk,  and  neck.  The 
head  and  upper  extremities  are  the  last  to  be  involved.     The  penis, 


108 


THE   DISEASES   OF   CHILDREN 


Fig.  19. 


scrotum,  soles  of  the  feet  and  palms  of  the  hands  remain  free.  The 
induration  increases  so  that  the  skin  gives  a  sensation  of  board-Uke 
resistance  and  coldness.  Various  descriptions  of  the  color  of  the  skin 
are  given  by  different  authors.  Neumann  describes  it  as  yellowish 
wliite  and  waxen;  Heubner  as  grayish  brown;  and  Parrot  as  light  blue 
and  cyanotic.  Small  ecchymoses  may  occasionally  be  found  on  the 
lower  extremities. 

The  skin  is  immovable  and  hard  and  cannot  be  raised  from  the 
underlying    tissue.      In   contradistinction   to   sclercedema   the   affected 

parts  become  atrophied.  The  legs  are 
rigid  and  the  cliildren  are  motionless 
like  a  stick  of  wood.  The  face  has  a 
mask-like  appearance. 

The  general  condition  is  bad,  the 
weight  falls  rapidly  and  the  tempera- 
ture is  constantly  sul)normal  and  may 
reach  30°  C.  (86°  F.)  or  lower.  Nur.s- 
ing  and  feeding  are  difficult  on  account 
of  the  rigidity.  The  mucous  mem- 
branes are  extremely  dry.  The  respi- 
ration is  lowered  to  16  or  even  less  a 
minute  and  the  pulse  falls  to  50  or 
30.  Older  children  will  give  a  shrill 
cry  (cri  dc  detresse)  at  frequent  in- 
tervals. Convulsions  may  occur.  The 
sleep  is  generally  disturbed.  The 
amount  of  urine  is  diminished  and  has 
a  heavy  sediment.  Albumin  is  absent. 
The  bowels  often  do  not  move  spon- 
taneously. The  child  generallj-  dies 
in  a  coma  after  a  few  days.  The  course 
is  the  more  rapid  and  fatal  the  younger 
the  infant.  The  author  has  had  cases 
of  infantile  atrophy  in  wliich  this  dis- 
ease ran  a  prolonged  course  lasting  weeks  and  ending  in  recovery. 

Occurrence. — Sclerema,  in  contrast  to  sclercedema,  is  a  very  rare 
affection.  It  occurs  not  only  during  the  first  days  of  Hfe  but  also  up  to 
the  first  three  months  (Berthod)  in  debiUtated,  premature  and  poorly 
nourished  infants.  Knopfelmacher  puts  the  age-limit  for  its  occurrence 
at  the  sixth  month.  It  is  observed  more  often  in  summer,  since  it 
occurs,  especially  in  older  nurslings,  as  a  sequelum  of  cholera  infantum 
and  chronic  catarrhal  enteritis  in  the  stage  of  atrophy  (Parrot, 
Henoch).  Congenital  cases  do  occur,  however,  although  with  ex- 
extreme  rarity. 


\ 


Sclerema  of  the  cutis  in  the  newborn.  Exten- 
sive thinning  of  the  epidermis  with  flattening  of 
the  papillae.  Diffuse  sclerosis  of  the  superficial 
layer  of  the  corium  and  hyaline  degeneration  of 
the  connective  tissue.  Partial  round-celled  in- 
filtration of  the  vessels  of  the  corium. 


SCLEIKEDEMA    AND    SCLEREMA  109 

Nature  and  Pathogenesis. — According  to  Luithlen  one  must  dif- 
ferentiate two  forms  of  .sclerema;  one  apparently  autochthonous,  that  is 
occurring  independent  of  any  other  disease;  hkewise  a  second  form 
developing  as  the  result  of  profuse  losses  of  plasma  or  as  a  consequence 
of  effusions  into  body-cavities  (pleuritis,  internal  bleedings).  This 
author  assigns  more  of  a  symptomatic  role  to  sclerema. 

Essentiall}'  the  process  consists  in  a  drying  up  of  Ihc  body  (Cle- 
mentowsky,  Widerhofer,  Soltmann).  The  peculiar  composition  of  the 
fat  in  the  newborn  and  young  nursling  according  to  the  investigations 
of  Langer  and  Knopfelmacher  seems  to  furnish  the  basis  for  the  oc- 
currence of  sclerema.  The  fat  of  a  newborn  contains  only  43.3  per  cent, 
of  oleic  acid  (Knopfelmacher)  against  6.5.0  per  cent,  in  the  adult  and 
in  older  cliildren  (Langer);  whereas  the  amount  of  palmitic  and  stearic 
acid  is  greater  (31:10)  in  the  young  nursling  and  congelation  takes 
place  at  a  loigher  temperature  than  in  older  children. 

Later  researches  (Tliiemich  and  Siegert)  have  left  these  findings 
again  in  doubt. 

The  lowering  of  the  external  temperature  must  play  an  etiolog- 
ical part  also  in  sclerema,  however  not  directl}'  through  the  effect 
of  the  cold  but  indirectly  by  unfavorably  affecting  the  respiration 
and  circulation.  Cases  occurring  without  previous  fluid  losses  and 
without  the  influence  of  cold  are  rare  and  etiologically  totally  obscure. 
A  few  authors  (Schmidt,  Aufrccht)  have  assumed  an  infectious 
origin  for  sclerema  although  the  adduced  bacterial  findings  only  go 
to  show  that  sclei"ema  can  occur  after  or  with  septic  disease  in  the 
newborn. 

The  hypothesis  of  a  vasomotor  and  trophic  vagus-neurosis  (llumesci 
d'Agata)  has  found  very  few  supporters. 

Pathological  Anatomy. — The  anatomical  findings  are  commonly 
negative.  The  usual  findings  are  only  those  of  atelectasis  in  the  new- 
born, or  else  the  evidences  of  a  more  or  less  acute  enteritis  in  older 
nurslings.  The  extreme  dryness  is  striking  as  well  as  the  hardness  of 
the  tissues,  on  section  of  the  skin  and  underlying  connective  tissue. 
The  adipose  tissue  is  firm,  dry,  stearin-like  and  resembles  a  piece  of  raw 
congested  fat  (Luithlen);  concerning  the  liistological  features  refer- 
ence is  made  to  the  illustration  (Fig.  19). 

The  diagnosis  is  made  from  the  board-hke  stiffness  of  the  skin 
together  with  the  markedly  abnormal  temperature  of  the  body.  The 
decrease  in  volume  of  the  members,  the  lack  of  the  sliiny  ajipearance 
of  the  slvin  as  well  as  the  failure  to  pit  serve  to  differentiate  tliis 
condition  from  scleroedema  (also  see  section  on  scleroderma).  Sclero- 
derma, which  usually  comes  in  older  and  stronger  children,  differs  from 
scleroedema  in  its  sharp  demarcation  and  Umitation  to  island-like  patches 
and  also  in  its  favorable  course  (Cruse,  H.  Neumann). 


110  THE    DISEASES   OF   CHILDREN 

The  prognosis  is  generally  bad  in  the  newborn,  but  somewhat 
better  in  older  children. 

The  prophylaxis  consists,  as  with  scleroedema,  in  the  avoidance  of 
sudden  and  violent  chilhng,  especially  with  debihtated  and  premature 
nurslings. 

The  treatment  resembles  that  of  sclercfidema  (q.v.)  in  many  re- 
spects. The  most  important  and  beneficial  measure  is  a  subcutaneous 
injection  of  salt  solution  [50-100  Gm.  (oiss-.5iii)  of  a  3d  1000  sodium 
chloride  solution].  This  can  be  injected  two  or  three  times  daily  or 
10  Gm.  (Siiss)  normal  salt  solution  [sodium  chloride  4  Gm.  (5i)  sodium 
bicarb.  3  Gm.  (gr.  xlv)  water  to  1000  Gm.  (1  quart)]  three  times  a  day, 
thoroughly  sterilized  and  given  at  a  temperature  of  40°-42°  C.  (104°- 
107.5°  F.).  High  rectal  injections  of  normal  salt  solution  can  be  used 
in  conjunction  with  the  subcutaneous  method. 


DISEASES  OF  PUBERTY 

BY 

Prof.  C.  SEITZ,  of  Munich 

translated   by 
Dn.  JOHN  ROWLAND,  New  York,  N.  Y. 


At  the  end  of  childhood,  even  before  growth  is  fully  attained  the 
human  being  is  capable  of  reproduction.  This  period  of  development  is 
known  as  puberty. 

The  chief  expression  of  beginning  sexual  power  is  the  developing 
specific  activity  of  the  embryonic  glands.  Spermatozoa  mature  in  the 
testicles  of  a  boy,  ova  in  the  ovaries  of  a  girl.  In  the  case  of  the  latter, 
the  fully  developed  ova  are  discharged  regularly,  about  every  4  weeks 
with  the  evidences  of  menstruation.  Nocturnal,  involuntarj^  discharges 
of  semen  begin  in  boys,  generally  before  they  are  completely  developed 
sexually;  these  take  place  at  first  at  long  intervals. 

Physiology. — Besides  these  events  which  constitute  the  nucleus 
of  the  development,  many  changes  are  to  be  observed  in  body  and  mind, 
the  sum  total  of  which  we  call  the  symptoms  of  puberty.  Striking 
changes  take  place  in  the  sexual  organs;  the  external  and  internal 
genitalia  become  more  vascular  and  grow  rapidly;  axillary  and  pubic 
hair  appear,  "Pubertas  a  pube  dicitur";  in  boys  the  beard  begins  to 
grow  and  hair  appears  on  the  rest  of  the  body,  especially  on  the  chest 
and  back.  The  figure,  which  up  to  this  time  has  been  childish,  changes 
in  a  typical  way,  the  shoulders  become  broader  because  the  thorax  in- 
creases greatly  in  breadth;  its  circumference  grows  in  3  years  about 
12  cm.;  the  maximum  capacity  of  the  lungs  often  increases  500  c.c. 
in  a  year.  The  breathing  in  females  becomes  more  and  more  costal  in 
type,  in  males  abdominal,  whereas  before  this  the  character  often  va- 
ried. In  addition  to  the  great  general  bodily  growth  which  attends  the 
development  of  puberty  the  sexual  type  becomes  more  apparent.  The 
breasts  of  girls  protrude  and  increase  in  consequence  of  the  development 
of  fat  and  connective  tissue,  moreover  the  branching  of  glandular  ducts 
proceeds,  though  glandular  tissue  capable  of  function  develops  only  in 
the  periphery  of  the  breasts.  The  female  pelvis  widens  in  all  dimensions 
and  the  thighs  and  buttocks  become  rounder  and  fuller  in  consequence 
of  a  marked  deposit  of  fat.  The  plastic  development  of  the  extremities 
in  youth  is  caused  by  a  growth  of  the  skeleton  but  also  by  an  increase 
in  the  size  and  firmness  of  the  muscles. 

Of  the  changes  in  the  inner  organs  that  of  the  larynx,  which  brings 

111 


112  THE   DISEASES   OF   CHILDREN 

about  the  change  of  voice,  is  first  to  be  mentioned.  This  is  in  general 
more  marked  in  boys,  in  whom  the  larynx  grows  rapidly,  especially  in  the 
transverse  diameter  with  corresponding  increase  in  length  of  the  vocal 
cords:  the  female  larynx  on  the  other  hand,  grows  more  in  the  vertical 
diameter.  Girls'  voices  break  less  often  than  boys  and  generally  only 
in  singing,  though  they  become  more  resonant  and  fuller.  Boys'  voices 
are  rough  and  harsh  and  after  a  time  of  frequent  breaking  Ijecome 
finally  an  octave  lower.  The  connection  between  change  of  voice  and 
sexual  development  is  evidenced  by  the  well-known  fact  that  boys, 
castrated  before  puberty,  show  a  decidedly  smaller  growth  of  the  larynx 
and  retain  very  high  voices.  The  dependence  of  the  development  of 
the  sexual  character  upon  the  growth  of  the'  embryonic  glands  can  be 
well  seen  in  these  individuals;  males  castrated  before  puberty  show  in 
addition  a  diminished  growth  of  beard,  slender  figures,  narrow  chests 
and  broad  pelves. 

The  connection  between  the  thyroid  gland  and  puberty  and  growth 
rests  upon  numerous  observations.  The  thyroid  gland,  in  which  a  con- 
gestive and  parenchymatous  swelling  occurs  before  and  during  puberty 
causing  an  increase  in  the  organ  of  15  per  cent,  and  at  the  time  of  men- 
struation frequently  60  per  cent.  (Fischer,  Freund),  has  also  a  decided 
influence  upon  bony  growth.  Thyreoidin  can  be  used  satisfactorily  to 
influence  a  cessation  of  this,  so  long  as  the  cartilages  are  not  calcified 
(Denis,  J.  J.  Schmidt).  Dysthyreoidiahas  not  only  a  restraining  influence 
upon  physical,  but  also  upon  mental  development  and  further,  the  special 
morphological  growth  of  the  genital  organs  of  both  sexes  and  the  de- 
velopment of  puberty  are  dependent  upon  the  normal  growth  and  func- 
tion of  the  thyroid  gland  (Hertoghe).  The  connection  between  the 
thyroid  gland  and  the  sexual  organs  is  shown  also  by  pathological  facts 
such  as  the  diminution  of  sexual  power  in  men  and  anomahes  of  mens- 
truation and  atrophy  of  the  sexual  organs  in  many  women  during  the 
course  of  Basedow's  disease,  the  development  of  which  not  infrequently 
dates  back  to  the  age  of  puberty  (Gra^^•itz).  If,  as  Hofmeister  says, 
the  thymus  gland  can  take  the  place  of  the  thyroid  gland  after  experi- 
mental extirpation  of  the  latter,  the  physiological  importance  of  the 
thymus  disappears  with  its  beginning  involution  which  takes  place 
often  long  before  the  child  enters  upon  puberty. 

Among  the  processes  of  growth  that  take  place  at  puberty  that  of 
the  heart  is  very  marked.  In  the  previous  years  this  has  remained  rel- 
atively small,  increasing  6  per  cent,  to  7  per  cent,  each  year,  but  at  pu- 
berty it  grows  rapidly  and  shows  an  increase  as  great  as  20  per  cent. 
The  considerable  growth  of  the  lungs  is  evident  from  the  figures  that 
have  been  mentioned  in  regard  to  the  circumference  and  capacity  of 
the  chest.  The  growth  of  the  Uver  and  kidneys  is  also  worthy  of  mention. 
The  brain  continues  to  grow  even  into  the  third  decade. 


DISEASES    OF    PUBERTY  113 

The  above-mentioned  changes  in  the  sexual  organs  that  serve  to 
indicate  puberty,  take  place  inside  a  period  of  one  to  two  years.  The 
general  bodily  development  as  regards  growth  and  increase  in  weight 
occupies  a  longer  time.  Upon  these  relations,  important  for  an  estima- 
tion of  the  physiology,  pathology  and  hygiene  of  later  childhood,  we 
possess  a  great  number  of  facts  which  have  been  obtained  through  the 
systematic  measurements  of  some  100,000  school  children  in  the  different 
countries  of  Europe  and  America.  According  to  Axel  Key  the  average 
growth  could  thus  be  decided  \\ith  certainty.  From  this  valuable  ma- 
terial the  follo^\^ng  facts  are  here  quoted:  the  increase  and  growth  in 
weight  and  length  exhibit  periodic  changes  and  do  not  follow  each  other 
closely;  growth  in  height  is  very  apt  to  precede  increase  in  weight.  In 
later  childhood,  before  puberty,  a  period  of  slow  growth  and  increase 
in  weight  occurs;  then  from  the  tenth  year  on  girls  begin  to  grow  more 
rapidly  and  this  growth  lasts  about  five  years  mth  its  ma.ximum  in  the 
twelfth  year.  An  increased  gain  in  weight  which  characterizes  the  more 
vigorous  time  of  puberty  lasts  from  the  twelfth  to  the  fifteenth  year. 
While  growth  generally  stops  at  the  seventeenth  year,  marked  increase 
in  weight  continues  as  late  as  the  twentieth  year.  In  the  case  of  boys  a 
decided  gro%yth  lasting  four  years  begins  in  the  fourteenth  year  and  the 
maximum  of  this  is  in  the  fifteenth  year.  The  weight  increases  mostly 
in  the  sixteenth  year.  The  sixteenth  and  seventeenth  years  are  the 
years  of  greatest  development  for  boys.  Boys  until  the  eleventh  year 
exceed  girls  in  length  and  weight,  the  latter  are  larger  until  the  sixteenth 
year  and  from  that  time  on  drop  behind  the  male  sex. 

The  strength-  of  build  is  generally  deiK-ndent  upon  the  develop- 
ment of  the  chest,  whose  circumference  stands  in  nearer  relation  to  the 
weight  than  to  the  length.  Children  who  have  grown  up  in  poor  sur- 
roundings remain  in  weight  and  length  constantly  behind  those  of  their 
own  age  in  better  circumstances.  The  period  of  slow  development 
noticeable  in  all  children  before  puberty  is  lengthened  in  the  poor,  in 
these  puberty  begins  later  but  this  stage  lasts  a  shorter  time  and  is 
ended  in  the  same  year  as  in  children  of  the  classes  better  situated. 

Besides  the  above-mentioned  effects  of  age  and  of  outward  circum- 
stances upon  the  bodily  development  of  the  child,  the  influence  of  the 
time  of  year  can  in  general  also  be  noticed,  in  so  far  as  during  the  winter 
months  a  shght  increase  takes  place,  in  spring  and  summer  a  growth 
in  height,  often  with  loss  in  weight,  and  finally  in  the  fall  decided  in- 
crease in  weight  results.  Likewise  changes  in  the  outward  temperature 
are  operative,  in  so  far  as  an  increase  in  temperature  at  any  time  of 
year,  even  though  it  lasts  only  a  few  days,  causes  an  increase  of  growth 
and  a  fall  in  temperature  causes  a  diminution  of  growth. 

In  general,  puberty  begins  in  girls  of  all  peoples  and  climates  earlier 
than  in  boys.     The  beginning  of  the  formation  of  semen  takes  place  in 

II -8 


114  THE   DISEASES   OF   CHILDREN 

the  latter,  in  the  majority  of  cases,  not  until  the  fifteenth  year,  while 
complete  maturity  is  generally  considered  to  be  reached  at  eighteen 
years.  The  age  at  which  boys  become  sexually  mature  and  girls  men- 
struate for  the  first  time  varies  under  the  influence  of  climate,  hered- 
itary predisposition,  race,  social  position,  method  of  fife,  and  individual 
pecuharity.  The  higher  the  mean  temperature  of  the  native  climate 
the  earlier  puberty  appears.  Menstruation  begins  in  Germany  most 
commonly  in  the  fourteenth  or  fifteenth  year  with  less  frequency  be- 
fore the  thirteenth  or  after  the  eighteenth  year.  It  is  earlier  in  girls 
living  in  cities  and  in  better  circumstances  than  in  those  living  in  the 
country  and  in  poorer  circumstances.  The  previous  use  of  alcohol  can 
through  the  excitation  of  sexual  desire,  cause  an  abnormally  early 
beginning  of  menstruation  and  premature  sexual  intercourse  has  the 
same  effect.  Girls  of  sanguine  temperament,  of  nervous  irritabihty,  of 
large  stature  and  of  strong  constitution  menstruate  earlier  than  phleg- 
matic individuals  or  than  small  or  weak  girls. 

The  arrival  at  maturity  shows  itself  in  children  by  various  manifes- 
tations that  are  noticeable  in  part  subjectively  and  in  part  objectively 
and  of  these  some  can  periodically  recur.  First  of  all  cardiac  palpita- 
tion is  commonly  noticed,  and  besides  this,  vertigo,  a  sense  of  oppression, 
shortness  of  breath,  nose  bleed  and  headache  are  not  infrequently  seen. 
(This  will  be  more  particularly  dealt  with  under  the  relation  of  puberty 
to  the  circulation.)  Especially  in  girls  one  sees  premonitorily  as  an 
expression  of  a  congestion  toward  the  genital  organs  an  occasional 
sense  of  pressure,  weight  and  twinges  in  the  lower  abdomen,  tender- 
ness in  the  region  of  the  ovaries  and  spasmodic  pain  in  the  abdomen 
and  lumbar  regions,  the  former  radiating  toward  the  epigastrium  and 
the  latter  toward  the  thighs.  Transient  and  even  painful  pricking  or 
tension  in  the  breasts  and  twinging  pains  along  the  ribs  are  common 
symptoms  present  oftener  in  girls  than  boys.  Besides  the  bodily  trans- 
formations that  indicate  puberty,  mental  changes  make  their  appear- 
ance. New  sensations  develop.  Boys  learn  from  emissions  the  specific 
sexual  sensations  out  of  which  an  impulse  to  attain  such  a  sensation 
can  develop.  The  awakening  of  the  sexual  impulse  is  dependent,  irre- 
spective of  the  progress  of  bodil}'^  development,  upon  the  exciting  in- 
fluences of  environment  (persuasion),  education  (sensual  excitation 
from  bad  hterature,  immoral  pictures),  and  food  (too  nourishing  food, 
the  use  of  alcohol).  We  \vi\\  speak  in  the  chapter  on  masturbation  of  the 
effect  of  an  abnormal,  hereditary  predisposition  and  of  the  irritation  of 
the  genital  nerves  by  phimosis  and  other  local  bodily  anomalies  on  the 
one  hand  and  of  early  provocation  of  the  sexual  sensation  on  the  other. 
Under  normal  circumstances  the  sexual  impulse  of  boys  first  shows  it- 
self only  in  occasional  erections  and  likewise  in  a  friendly  affection  for 
girls,  provided  that  the  company  of  girls  is  not  at  first  despised.     Emis- 


DISEASES    OF   PUBERTY  115 

sions  and  such  acts  do  not  take  place  in  normal  girls  and  to  them  at  first 
sexual  sensations  are  still  foreign  (LiJwenfeld);  on  the  other  hand 
specific  female  characteristics  show  themselves,  such  as  solicitude  for 
younger  brothers  and  sisters  and  tenderness  for  strange  children.  In 
addition  young  girls,  as  is  well  known,  readily  ideahze  a  teacher,  an 
officer,  favorite  actors,  etc.  In  general  the  mental  changes  in  maturing 
girls  take  place  more  quietly  than  in  boys  in  whom  the  growing  sense 
of  manhood  often  finds  expression  in  an  increased  sense  of  importance 
and  an  arrogant  disposition  (EmminghaUs).  In  consequence  of  tlus 
change  of  disposition  we  notice  more  often  in  boys  than  in  girls  a  pecu- 
liar transformation  of  the  mental  and  bodily  power  that  characterizes 
these  years.  This  shows  itself  in  chaffing  and  teasing  of  those  younger 
and  weaker,  derision  of  the  infirm,  cruelty  to  animals  and  foolish  acts 
of  all  kinds.  Also  against  parents  and  teachers  a  sense  of  superiority 
is  felt;  their  warnings  are  laughed  at  and  the  reaction  against  them 
amounts  to  insubordination  and  even  rising  animosity.  Falsehood  and 
bragging,  sentimentahty  and  extravagance  are  e\'idences  of  the  fre- 
quent mental  variations.  Depression  and  buoyancy  abruptly  alternate. 
This  unsteadiness  of  mind  is  evidenced  by  the  often  clumsy,  awkward 
and  clownish  movements  of  the  rapidlj^  growing  body.  The  rapidity  of 
the  mental  growth  is  subject  to  great  variations,  sometimes  the  intelli- 
gence develops  more  rapidly,  at  other  times  the  emotions  alone.  Bodily 
and  mental  progress  do  not  take  place  throughout  in  parallel  lines,  it  is 
more  common  for  the  mental  development  to  stand  still  when  bodily 
growth  is  accelerated.  The  psychical  changes  mentioned  above  con- 
tinue beyond  the  time  of  bodily  development  and  only  in  the  eighteenth 
year  or  later  is  the  boy  relieved  of  this  disturbing  experience.  It  must  be 
confessed,  however,  that  from  tills  time  permament  psychical  distur- 
bances may  date. 

Pathology. — Puberty  may  be  premature  or  delayed.  Under  the 
head  of  premature  development  we  see  an  early  mental  and  bodily 
development  going  hand  in  hand  or  independently  following  one  an- 
other. The  early  bodily  development  usually  consists  in  a  more  rapid 
growth  of  the  body  without  corresponding  involvement  of  the  sexual 
organs  but  less  frequently  sexual  maturity  is  independent  of  and  pre- 
cedes bodily  development  (Kussmaul).  There  are  numerous  observa- 
tions of  menstruatio  pra?cox  (before  the  tenth  year)  upon  record  even 
in  children  during  the  first  year,  in  whom  in  addition  to  a  dispropor- 
tionate development  of  the  breasts  and  outer  genitaha  regular  bleeding 
took  place  usually  at  considerable  intervals.  Menstruation  in  these 
cases  is  however  rare.  Pregnancies  progressing  in  a  relatively  normal 
manner  have  also  been  recorded  as  early  as  the  eighth  or  ninth  year. 
This  early  menstruation  is  referred  to  congenital  errors  in  development 
with  premature  bodily  growth  as  its  result,  to  excessive  maternal  pro- 


& 


no  THE   DISEASES   OF   CHILDREN 

ductiveness,  to  diseased  conditions  in  tlie  ova,  to  irritation  of  the  trophic 
centres  (hydrocephalus,  shock)  and  to  sexual  excitation.  In  those 
who  mature  early,  bodily  growth  generally  ends  with  complete  sexual 
maturity.  Early  maturity  is  sometimes  combined  with  enormous  fat 
formation  which  latter  can  appear  early  by  itself  or  can  accompany  a 
precocious  bodily  growth  without  early  sexual  power.  Girls  may  show 
a  sexual  prematurity  alone;  but  in  boys  this  early  sexual  development 
generally  goes  hand  in  hand  with  an  early  development  of  the  whole 
body.  Emissions  of  semen  have  been  noticed  in  such  boys  very  early, 
from  the  second  year  on.  The  mental  growth  does  not  progress  parallel 
with  the  bodily  development  in  those  early  matured,  it  seldom  precedes 
it,  usually  it  takes  place  later. 

Amcnorrhoea  can  be  simulated  by  atresia  of  the  uterovaginal 
canal;  in  consequence  of  the  collection  of  blood  behind  the  atresia  with 
distention  of  the  vagina,  uterus  and  tubes,  ha:>matocolpus,  lupmatometra 
or  hicmatosalpinx  may  arise  (Gebhardt).  In  consequence  of  congenital 
heart  disease  (pulmonary  stenosis)  there  may  be  a  deficient  develop- 
ment of  the  genitalia.  AVith  congenital  aplasia  of  the  ovaries,  the  uterus 
is  also  insufficiently  developed  and  ovulation  and  menstruation  are 
absent.  Menstruation  can  hardly  take  place  if  functionating  ovaries  are 
combined  \vith  a  rudimentary  uterus  but  it  may  evidence  itself  in  the 
form  of  pains  and  nervous  disturbances  recurring  periodically  and  with 
intensity.  A  foetal  uterus  can  be  suspected  if  the  breasts  do  not  develop 
or  if  the  pubic  hair  fails  to  appear  at  the  proper  time.  Errors  in  the  de- 
velopment of  the  inner  genitalia  in  girls  are  first  clinically  noticeable  at 
the  time  of  puberty,  but  even  before  this  deficient  development  of  the 
ova  causes  a  retardation  of  bodily  growth.  Functional  amenorrhoea  in 
the  years  of  development  is  not  infrequently  noticed  after  a  sudden 
change  of  social  or  climatic  conditions  and  further,  in  consequence  of 
constitutional  anomalies  such  a  chlorosis,  anemias  of  all  kinds,  after 
acute  infectious  diseases,  in  tuberculosis,  severe  sypliihs,  nephritis, 
neuroses,  psychoses,  diabetes,  leukaemia,  Basedow's  disease,  alcohoHsm 
and  morpliinism.  When  menstrual  bleeding  fails  or  is  deficient  in  quan- 
tity even  at  the  age  of  puberty  the  appearance  of  periodically  recurring, 
vicarious  bleeding,  from  the  nose,  gums,  lungs,  stomach,  hemorrhoids, 
nipples  and  ears  is  sometimes  seen. 

Independent  of  the  local  premonitory  disturbances  that  have  been 
previously  mentioned  (p.  114)  we  sometimes  find  even  at  the  first  men- 
struation prodromal,  concomitant  or  succeeding  cramp-like  abdominal 
pains  (especially  in  the  case  of  nervous  or  chlorotic  girls  or  with  hypo- 
plasia of  the  uterus).  In  connection  with  the  nervous  system,  vertigo 
or  intense  headache  may  be  noticed;  functional  stomach  and  intestinal 
disturbances  (such  as  regurgitation,  vomiting,  cardialgia,  diarrhoea, 
constipation  or  flatulence)  can  appear.     The  congestive  sacral  pains  are 


DISEASES    OF   PUBERTY  117 

sometimes  accompanied  bj'  a  desire  for  urination  or  defecation.  At  tlie 
age  of  puberty  reflex  angioneuroses  arc  also  found.  Besides  tiie  pre- 
menstrual sensations  of  heat  and  cold,  erythemata,  urticaria,  eruptions 
of  herpes  are  noticed  prodromally  or  concomitantly  and  further  as  a 
forerunner  periodic  painful  ccdema  of  the  extremities  may  be  found. 

The  revolution  which  at  the  time  of  puberty  attacks  especially  the 
sexual  organs,  often  affects  the  male  breasts.  The  atrophy  of  these 
generally  takes  place  at  this  time  w-ithout  sj^mptoms.  Nevertheless,  a 
sensitive  swelling  of  one  or  both  breasts  may  sometimes  occur  with  red- 
dening and  pigmentation  of  the  nipple  accompanied  by  first  dull  and 
later  sharper  pain.  These  sensations  are  often  referred  to  trauma 
and  disappear  spontaneously  in  the  course  of  a  few  weeks.  They  may 
however  recur,  but  the  l)reasts  rarely  remain  enlarged  (gynecomastia) 
in  which  case  a  deficient  growth  of  the  genitals  would  also  be  noticed. 
In  both  sexes  at  puberty  a  circumscribed  nodular  swelling  of  the  breasts 
can  be  caused  by  a  growth  of  interstitial  connective  tissue  but  this  also 
disappears  later  on  and  seldom  is  it  the  point  of  origin  of  a  new  growth. 

In  the  male  genitals  unpleasant  sensations  sometimes  occur  because 
of  an  incomplete  descensus  testiculi  which  up  to  that  time  had  not  been 
observed.  AVith  retentio  testis  inguinalis  (less  commonly  with  the 
abdominal  or  perineal  variety)  boys  complain  sometimes  of  drawing 
pains  in  the  testicle  (or  spermatic  cord).  Sharper  pains  during  puberty 
are  caused  especially  by  inflammatory  irritation  which  occurs  readily 
after  injuries  (gymnastic)  or  after  mumps,  etc.,  in  consecjuence  of  the 
confined  position  of  the  retained  testicle.  As  retained  testicles  not  in- 
frequently become  atrophic  and  further  show  a  tendency  to  mahgnant 
degeneration,  removal  of  the  testicles  is  to  be  considered  provided  a 
hernia  pad  especially  constructed  and  to  be  worn  \nthout  intermission 
does  not  hold  the  testicle  constantly  pressed  below  it  (Heidenhein). 
Analogous  to  the  displaced  testicles  of  boys  changes  in  position  of  the 
ovaries  (ovarian  hernias)  are  apparent  in  girls  especially  at  puberty, 
through  periodic  swelling  of  this  organ  (Hennig).  At  puberty  not  in- 
frequently cases  of  pseudohermaphroditism  can  be  determined  as  to 
sex,  as  retained  testicles  pass  into  the  sides  of  the  .scrotum  separated  by 
an  extreme  hypospadias  and  in  addition  changes  follow  in  the  charac- 
teristics of  the  child  which  up  to  that  time  seemed  to  be  feminine. 
Phimosis  also  can  give  rise  at  puberty  to  manifold  disturbances.  At  this 
time  especially,  a  quantity  of  readily  decomposing  smegma  is  produced 
whose  retention  leads  to  balanitis  and  balanoposthitis  with  a  foul 
smelHng  discharge  which  can  simulate  gonorrhoea.  A  microscopical 
examination  of  the  secretion  for  gonococci  prevents  error.  Successful 
treatment  is  only  possible  after  operative  removal  of  the  phimosis;  this 
is  further  to  be  considered  when  -n-ith  a  narrow  prepuce  enuresis  or 
masturbation  comes  on  at  puberty. 


118  THE    DISEASES   OF   CHILDREN 

The  chief  development  of  a  sj'stematic  practice  of  onanism  occurs 
(according  to  Fiirbringer)  at  puberty.  Provided  that  this  bad  habit 
has  not  ah-eady  been  practiced,  a  local  irritation  of  the  genital  nerves 
(through  eczema,  prurigo,  phimosis  or  vulvitis  ^ith  an  accumulation  of 
smegma,  oxyurides,  stone  in  the  bladder,  constipation)  plays  the  part 
of  an  exciting  cause  in  connection  with  the  awakening  sexual  passion 
(see  page  114).  This  complaint  is  more  often  the  outcome  of  a  neuro- 
pathic dispo.sition  with  hereditary  weakness  of  mil  than  the  result  of 
an  excessive  hereditary  sexual  desire  (Lowenfeld).  Idiots,  those  men- 
tally deficient,  and  epileptics  often  show  a  decided  tendency  to  onanism. 
In  boarding  schools  (occasionally  breaking  out  epidemicall}'  in  conse- 
quence of  living  \\\{\\  certain  depraved  scholars)  the  evil  is  commoner  in 
boys  than  in  girls.  Provided  there  is  no  excessive  practice  of  this  evil 
habit,  striking  symptoms  on  the  side  of  the  nervous  system  are  not  to 
be  found  in  otherwise  healthy  individuals.  But  one  sees  in  habitual 
masturbators  in  addition  to  ansemia  and  an  exhausted  appearance  not 
infrequenth^  the  signs  of  general  nervousness  with  migraine,  sense  of 
pressure  in  the  head  or  of  cardiac  neuroses,  with  palpitation  and  sense 
of  pressure  or  spinal  s^'mptoms  (exhaustion,  parresthesia  in  the  legs, 
sacral  pains  and  other  annoying  sensations  in  the  back).  The  associa- 
tion during  the  period  of  development  of  intense  mental  effort  with 
excessive  onanism  injures  the  brain  in  its  power  of  resistance  and  accom- 
plishment; beside  loss  of  energy,  lack  of  memory,  indisposition  and 
incapacity  to  work,  absent-mindedness  is  often  a  striking  symptom. 
Sexual  neurasthenia  not  infrequently  begins  at  puberty.  The  e\'il  re- 
sults of  masturbation  can  be  completely  overcome  by  early  and  rational 
opposition.  Positive  local  signs  in  the  genitalia,  apart  from  slight  evi- 
dences of  irritation,  are  not  to  be  determined.  For  the  purpose  of  pro- 
phylaxis the  above-mentioned  bodily  anomalies  that  lead  to  irritation 
of  the  genital  organs  are  to  be  removed;  of  importance  also  is  food 
causing  as  httle  irritation  as  possible  (especially  the  avoidance  of  alcohol). 
Reading  and  companions  should  be  overseen,  often  severe  bodily  exer- 
cise out  of  doors  is  of  advantage  and  further  the  awakening  of  a  mental 
interest  sometimes  by  travelling.  If  the  evil  persists  the  moral  influence 
of  parents  and  teachers  is  often  of  value  in  addition  to  these  other 
measures;  there  is  less  to  be  accomplished  by  severe  punishment  than 
there  is  by  kindly  explanation,  if  necessary  with  the  assistance  of  the 
physician.  If  on  the  one  hand  as  can  be  seen  by  the  foregoing  descrip- 
tion, sexual  development  gives  rise  to  most  manifold  disturbances,  so 
on  the  other  hand  at  puberty  occasional  functional  disturbances  occur 
in  almost  all  the  organs  which  may  often  have  sweeping  and  enduring 
consequences,  and  the  general  health  at  this  time  is  subject  to  great 
variations.  A  consideration  of  the  ratio  of  disease  during  this  age  of 
development  gives  us  the  following  striking -facts.     We  gather  from  the 


DISEASES   OF    PUBERTY  119 

statements  of  Axel  Key,  Hansen,  Hertel  and  others  which  claim  the 
greatest  possible  accuracy,  that  in  the  middle  grade  schools  the  general 
sickness  of  boys  reaches  nearly  40  per  cent,  and  of  girls  60  per  cent,  and 
over.  While  by  the  latter  antpmia  amounts  to  40  per  cent.,  by  the  former 
myopia  occupies  the  first  place.  Sixteen  per  cent,  of  boys  suffer  from 
chronic  headache,  36  per  cent,  of  girls,  of  the  latter  10  per  cent,  have 
spinal  curvature.  Of  chronic  diseases  it  has  been  determined,  that  those 
of  the  lungs  amount  to  3k  per  cent.,  of  the  heart  3  per  cent.,  and  of  the 
gastro-intestinal  tract  2h  per  cent.  From  personal  observations  of 
2500  cases  observed  in  the  last  four  years  in  the  pediatric  polyclinic  and 
of  500  from  private  practice,  260  suffered  from  acute  infectious  diseases, 
354  from  tuberculosis,  7  from  syphilis,  325  from  aniemia  and  chloro.sis, 
350  from  diseases  of  the  respiratory  organs,  580  from  diseases  of  diges- 
tion, 90  from  diseases  of  the  urogenital  organs,  280  from  skin  diseases 
and  60  from  organic  heart  disease.  Disturbances  were  found  in  600 
cases  which  could  be  brought  into  more  or  less  close  connection  with 
puberty.  Of  the.^e  22  per  cent,  had  general  evidences  of  menstruation, 
dra^-ing  and  other  kinds  of  pains  in  the  breasts  or  abdomen  without 
objective  physical  signs;  16  per  cent,  had  cardiac  neuroses,  16  per  cent, 
goitre,  16  per  cent,  periodic  headaches,  15  per  cent,  neurasthenia,  15 
per  cent,  hysteria,  7  per  cent,  epilepsy,  4  per  cent,  chorea  and  6  per 
cent,  the  albuminuria  of  puberty.  Of  the  chronic  infectious  diseases, 
tubercidosis  at  this  critical  period  is  of  the  utmost  significance  on  ac- 
count of  its  great  frequency  and  its  often  fatal  result.  According  to 
Kirchner,  between  the  ages  of  ten  and  fifteen,  the  deaths  from  tubercu- 
losis in  males  increase  from  ten  to  sixteen  in  a  hundred  deaths  and  in 
females  from  eighteen  to  twenty-six  in  a  hundred.  After  tuberculosis 
the  anomalies  of  the  lilood — especially  chlorosis — take  the  first  place. 
As  far  as  the  diseases  of  the  single  organs  are  concerned  those  of  the 
respiratory  and  digestive  tract  are  not  infrequent  and  affections  of  the 
skin  still  show  a  great  frequenc}'.  Functional  disturbances  are  found  in 
20  per  cent,  of  children  at  puberty  according  to  my  observations  and 
these  are  to  be  referred  to  the  processes  of  development;  three-fifths  of 
these  affect  females  and  two-fifths  affect  males. 

Let  us  now  turn  to  the  diseases  of  the  special  organs  or  systems  in 
so  far  as  they  are  made  evident  during  puberty  by  special  symptoms  or 
by  great  frequency.  In  the  increased  general  bodily  growth  which 
accompanies  puberty  the  bony  and  muscular  systems  are  chiefly  involved 
and  the  diminished  resistance  (insufficient  firmness  of  the  skeleton  and 
relative  weakness  of  the  muscles)  which  occurs  in  consequence  of  this 
gives  rise  to  the  most  various  disturbances.  In  the  first  place,  curva- 
tures of  the  spine — especially  lateral  curvatures — occur  at  this  time  in 
consequence  of  customary  but  impro]ier  attitudes  at  school  and  in 
housework;    further,    in   consequence    of   unilateral    muscular    exercise 


120  THE   DISEASES   OF   CHILDREN 

(for  example  violin  playing  and  tennis)  and  in  consequence  of  unilateral 
loading  of  the  body  (the  carrying  at  the  side  of  heavy  school  bags,  etc.). 
Genu  valgum  can  also  develop  at  this  age  by  reason  of  prolonged  stand- 
ing. The  changes  that  have  been  mentioned  are  the  more  easily  pro- 
duced according  as  bodily  exercise  has  been  disregarded  in  consequence 
of  an  education  devoted  too  exclusively  to  mental  improvement  and 
further  when  by  reason  of  improper  food  at  this  critical  time  the 
composition  of  the  blood  becomes  deficient  or  was  deficient  from  the 
beginning.  The  possibility  of  late  rickets  has  recently  been  emphasized 
from  the  surgical  side  (Roos,  H.  Curschmann)  as  a  cause  for  genu  val- 
gum and  also  for  curvatures  of  the.  spine  or  for  flattening  of  the  pelvis 
in  so  far  as  these  anomalies  develop  during  puberty.  With  the  increased 
processes  of  ossification  that  go  on  at  this  time  analogous  to  those  at 
the  infantile  period  of  bony  formation  a  special  disposition  to  rachitic 
disease  is  supposed  to  exist.  At  this  stage  of  rapid  growth  a  slight 
amount  of  bodily  fatigue  easily  occurs  and  forced  exertion  undertaken 
concurrently  with  stronger  comrades,  especially  in  sports,  gives  rise  to 
overexhaustion.  This  shows  itself  generally  in  addition  to  a  general 
weakness  in  a  somewhat  diminished  motility  of  the  joints  and  in  pain 
on  pressure  over  the  epiphyses  chiefly  involved  in  growth  without  other 
objective  changes.  These  are  the  upper  epiphyses  of  the  humerus 
and  tibia  and  the  lower  epiphyses  of  the  radius  and  femur.  The  pains 
which  generally  disappear  rapidly  are  described  as  growing  pains  (espe- 
cially in  France  by  Poncet,  Bouilly,  Comby)  and  growing  fever  is  also 
described.  These  are  statements  which  we  should  accept  with  caution. 
Certain  it  is  that  at  this  time  pain  in  the  extremities  results  from  slight 
trauma  or  overexertion  and  also  with  rheumatic  and  other  febrile  in- 
fections following  which  as  is  well  known  a  more  rapid  growth  often 
occurs  (poussee  de  croissance). 

Finally  the  multiple  cartilaginous  exostoses  developing  only  on 
bones  still  in  the  process  of  growth  and  which  occur  from  an  inherited 
source,  should  be  mentioned  in  this  place.  These  generally  appear  as 
symmetrical,  hard,  painless  indurations  and  rough  tumors  in  the  neigh- 
borhood of  the  epiphyses  from  which  they  may  be  separated  in  the 
process  of  growth. 

Of  the  myopathic  muscular  atrophies  the  infantile  muscular  dys- 
trophy with  pseudohypertrophy  (of  the  calves,  thighs,  gluteal  and 
deltoid  muscles)  and  the  infantile  muscular  hypertrophy  (without 
pseudohypertrophy)  mth  involvement  of  the  muscles  of  the  face  are 
likely  to  develop  before  puberty.  During  the  period  of  puberty  the 
so-called  juvenile  form  of  muscular  atrophy  appears  with  the  initial  in- 
volvement of  the  shoulder-girdle  muscles  and  later  the  muscles  of  the 
upper  arm  (with  the  exception  of  the  deltoid,  supra-  and  infraspinatus 
and  coracobrachialis)  and  finally  the  nmscles  of  the  back  and  pelvis. 


DISEASES    OF    PUBERTY  bil 

Anomalies  of  the  blood  are  a  decidedly  common  phenomenon  at 
the  age  of  puberty.  These,  so  far  as  the  anteniias  are  concerned,  date 
from  some  previous  time  or  occur  very  easily  in  consequence  of  severe 
acute  infectious  diseases,  protracted  fevers  following  irrational  methods 
of  life  (improper  food,  athletic  exhaustion)  or  unhygienic  living  or  work- 
ing conditions.  Tuberculosis,  kidney  affections,  constipation  and  entozoa 
are  always  to  be  thought  of.  An  absolute  predisposition  for  chlorosis 
exists  in  the  female  sex,  the  blood  of  which  is  from  the  beginning  poorer 
in  red  blood  cells  by  nearly  half  a  miUion  per  cmm.  but  besides  this 
(according  to  Jones  following  Grawitz)  it  shows  especially  at  puberty  a 
relatively  smaller  hsemoglobin  content.  The  number  of  leucocytes  is 
higher  at  puberty,  9-12000  per  cmm.  (Bayer).  For  a  further  considera- 
tion of  chlorosis  see  the  chapter  on  blood  diseases. 

In  the  circulatory  system,  disturbances  frequently  arise  at  puberty 
in  the  form  of  cardiac  palpitation,  sensations  of  pressure,  vertigo, 
syncope  and  shortness  of  breath.  Only  when  alcohol,  nicotine,  kidney 
disease  and  overexertion  can  be  excluded  as  causes,  is  a  direct  connec- 
tion with  puberty  to  be  considered.  Objectively  one  finds  an  enlargment 
of  the  right  or  left  side  of  the  heart  (sometimes  both  sides)  with  a  soft 
pulse  and  an  apex  beat  which  may  be  exaggerated  without  an  evident 
increase  in  size  of  the  heart  and  this  generally  with  an  accentuated 
second  aortic  sound  and  often  with  an  accentuated  second  pulmonic 
sound.  Sometimes  the  sounds  are  ringing  in  character.  The  tension  is 
seldom  increased  and  the  arteries  rarely  tortuous.  Systolic  murmurs  at 
the  apex  or  in  the  second  left  intercostal  space  are  heard  with  and  with- 
out an  accentuated  second  pulmonic  sound.  The  pulse  is  increased  in 
frequency  and  may  sometimes  be  irregular.  An  excited  heart  action 
at  the  time  when  various  organs  show  an  increased  excitabiUty  can  be 
simply  evidence  of  irritation  (Krehl)  but  generally  there  is  an  actual 
disproportion  between  the  development  of  the  chest  and  the  size  of  the 
heart,  a  decided  increase  in  the  cardiac  volume  and  a  relative  narrowness 
of  the  blood  vessels;  a  further  disproportion  can  also  occur  between  the 
rapid  expansion  of  the  blood  vessels  in  consequence  of  a  sudden  growth 
in  length  (especially  after  afebrile  diseases)  and  an  undeveloped  heart 
which  cannot  meet  the  increased  demands  made  upon  it.  An  insuffi- 
cient growth  of  the  heart  generally  goes  hand  in  hand  with  a  retarded 
growth  of  the  whole  body  and  especially  of  the  sexual  organs:  hypo- 
plasia of  the  heart  is  generally  congenital  as  is  also  an  especial  narrow- 
ness of  the  large  blood  vessels,  but  this  nevertheless  frequently  manifests 
itself  only  at  puberty  (Berg).  The  subjective  and  objective  phenomena 
which  have  been  mentioned  begin  without  any  apparent  outward  cause, 
continue  for  a  long  time  with  more  or  less  regularity  or  may  periodically 
increase,  especially  the  subjective  symptoms.  A  complete  disappearance 
of  the  cardiac  symptoms  generally  follows  the  completion  of  puberty, 


1-22  THE    DISEASES   OF   CHILDREN 

by  virtue  of  a  readjustment  of  the  cquilibriuni.  Not  so  very  infre- 
quently an  exaggerated  apex  beat  remains  permanent  and  this  may  also 
be  true  of  a  functional  weakness  wliich  shows  itself  later  on  in  frequent 
attacks  of  weakness,  of  syncope,  etc.,  follomng  slight  exertion  or  excite- 
ment. The  prognosis  of  the  cardiac  disturbances  at  puberty  is  to  be 
made  ^\'ith  caution,  as  transient  changes  in  the  size  of  the  heart  are  not 
always  to  be  distinguished  from  definite  changes  in  the  muscle.  Pro- 
phylactically  and  therapeutically,  caution  is  to  be  exercised  against  the 
influences  wliich  can  injure  the  heart,  such  as  excessive  meat  eating, 
alcohol,  nicotine,  coffee,  tea,  sexual  excitement  and  especially  against 
athletic  overexertion.  When  on  account  of  the  increased  growth  of  the 
body,  the  heart  must  accomodate  itself  to  increased  demands,  a  nutri- 
tious non-irritating  diet  is  necessary  as  well  as  sufficient  bodily  rest  and 
exercise  corresponding  to  the  patient's  present  condition.  Tliis  can  best 
be  accomplished  by  moderate  out-of-door  exercise,  walking,  etc.  Chil- 
dren with  valvular  disease  not  infrequently  show  at  puberty  extreme  car- 
diac disturbances  on  account  of  the  association  of  functional  disorders 
with  the  valvular  lesions. 

The  thyroid  gland  whose  connection  ■udth  puberty  has  already  been 
mentioned  (p.  112)  shows  not  infrequently  a  strumous  degeneration  in 
addition  to  the  temporary  or  periodic  conjestive  tumefaction  which 
has  been  noted.  This  is  sometimes  noticed  in  earhest  childhood  on  the 
basis  of  an  inherited  predisposition  but  is  most  commonly  observed  in 
the  fourteenth  or'  fifteenth  year  or  in  the  two  following  years.  It  is 
more  frequent  in  females  in  whom  this  gland  is  from  the  beginning  more 
developed  (Demme).  While  the  physiological  congestion  is  transitory 
in  puberty,  mechanical  causes  at  this  time  can  give  rise  to  a  chronic  con- 
gestion; these  may  be,  carrying  heavy  weights  on  the  head  and  neck,  ex- 
cessive singing  exercises,  tight  bands  around  the  neck;  the  congestion  may 
also  be  influenced  by  pertussis,  chronic  pneumonia  and  valvular  lesions. 
For  the  different  forms  of  goitre  see  the  article  by  Siegert  in  Vol.  III. 

There  are  no  affections  of  the  respiratory  system  that  stand  in 
close  connection  with  puberty.  The  expectation  that  has  been  cherished 
by  some  that  adenoid  vegetations  of  the  nasopharynx  will  disappear  at 
this  time  or  cause'no  more  symptoms,  on  account  of  the  increase  of  the 
pharynx  progressing  parallel  with  the  increased  bodily  growth,  is  not 
fulfilled.  Operative  removal  of  the  growths  should  be  strongly  advised 
in  order  that  their  continuance  may  not  interfere  with  the  permanent 
expansion  of  the  chest. 

With  the  change  of  voice  that  has  been  described  on  page  111,  it 
frequently  happens,  more  commonly  in  boys,  that  the  voice  is  incor- 
rectly developed.  It  may  remain  high  or  crowing,  it  often  fails  or  cracks. 
In  consequence  of  the  rapidly  following  changes  in  the  larynx  in  form 
and  size,  the  proper  sensation  for  the  changing  conditions  of  tension  is 


DISEASES    OF   PUBERTY  123 

lost  (Bresgen).  Relative  quiet  for  the  voice  (abstinence  from  singing 
or  shouting)  is  prophylactically  of  value,  for  therapeutic  purposes,  prac- 
ticing in  a  moderately  loud  voice  with  the  deepest  tones  and  possibly 
with  sHght  compression  of  the  larynx,  is  effective.  In  children  who 
previously  have  had  a  tendency  to  diffuse  bronchitis  possibly  with  dys- 
pnoea, one  may  see  typical  attacks  of  asthma  occur  if  at  the  same  time 
with  the  rapid  increase  in  length  that  accompanies  puberty,  a  delayed 
development  of  the  thorax  is  present.  These  attacks  of  asthma  can 
disappear  entirely  when  growth  is  completed  (Miiller). 

In  so  far  as  the  digestive  system  is  concerned,  frequently  recurring 
attacks  of  tonsilhtis  play  a  considerable  part  in  the  morbidity  at  this 
time;  these  attacks  occur  generally  in  individuals  already  predisposed 
to  them.  Various  dyspeptic  complaints  are  also  seen  wliich  are  charac- 
terized by  their  periodic  recurrence,  and  in  the  absence  of  an  irrational 
diet  as  a  cause  they  are  to  be  con.sidered  as  nervous  symptoms.  Not 
infrequently  gastroptosis  and  enteroptosis  begins  in  these  years,  espe- 
cially in  females,  influenced  by  constricting  clothes,  in  connection  with 
an  irrational  and  excessive  amount  of  food  (Meinert).  Ulcer  of  the 
stomach,  which  is  almost  never  found  in  childhood,  occasionall}'  occurs 
at  puberty.  Functional  motor  disturbances  of  the  intestine  are  decidedly 
common  at  this  time;  especially  in  girls  chronic  constipation  is  noticed 
and  with  this  one  has  to  contend  not  only  with  insufficient  exercise  and 
unsuitable  food  but  often  with  a  false  sense  of  modest}'.  The  abnor- 
mally long  retention  of  feces  or  urine  which  not  infrequently  takes  place 
in  school  girls  can  cause  an  ante-  or  retroflexion  of  the  uterus  (Hennig). 
Occasionally  periodic  attacks  of  charrhoca  are  observed  that  cannot  be 
explained  by  errors  in  diet;  less  commonly  a  sudden  desire  for  stool 
with  incontinence  occurs,  of  a  temporary  congestive  or  nervous  origin. 
Before  the  beginning  of  this  period  Quincke  saw,  in  an  otherwise  health}^ 
girl,  twelve  to  fourteen  years  of  age,  an  ascites  slowly  develop  which 
disappeared  rapidly  at  the  beginning  of  menstruation. 

Diseases  of  the  urinary  organs  are  not  common  in  pulierty;  still 
the  majority  of  cases  of  cyclic  or  orthostatic  albuminuria  begin  at  this 
period  so  that  one  can  speak  of  albuminuria  of  puberty.  The  patients 
are  often  pale,  tall,  and  slim  individuals  easily  tired*  with  swollen  eye 
lids  and  a  tendency  to  headache,  vertigo  and  dyspeptic  complaints.  The 
disturbances  of  the  heart  which  have  l)een  mentioned  on  page  121.  are 
frequently  present.  But  in  otherwise  healthy  indi%-iduals  at  puberty 
one  finds  a  periodic  excretion  of  albumin,  sometimes  more,  sometimes 
less,  in  diminishing  quantity  in  the  night  urine.  This  occurs  often  with 
uneven  growth  and  with  a  backward  general  development.  The  urine 
shows  a  high  specific  gravity;  sediment  is  absent  (or  a  few  fatty  epithe- 
lial cells  and  hyaline  casts);  chemically  the  demonstration  of  an  albu- 
min precipitable  by  acetic  acid  (euglobulin)  is  important,  for  this  in  the 


124  THE   DISEASES   OF   CHILDREN 

chronic  nephritis  of  cliildren  is  a])sent  or  only  present  in  traces  (Lang- 
stein).  From  the  standpoint  of  differential  diagnosis  one  must  always 
consider  the  exclusion  of  such  nephritides  which  can  begin  relativeh' 
without  symptoms  especially  in  conjunction  with  infectious  diseases 
and  may  persist  insidiously.  The  albuminuria  of  puberty  disappears 
when  the  orgajiism  of  the  affected  individual  recovers  its  balance  in  con- 
sequence of  better  formation  of  blood  and  better  nourishment  with  the 
completion  of  growth  (in  individuals  with  a  congenital  abnormal  per- 
viousness  of  the  kidneys,  the  disposition  to  an  excretion  of  albumin  can 
remain  permanent).  Therapeutically  a  rest  cure  is  not  always  success- 
ful; in  place  of  tliis  properly  graded,  systematic  exercise  with  a  view 
to  greater  general  and  especially  cardiac  development  should  be  em- 
ployed. Very  exhausting  bodily  exertion  should  be  rigidly  excluded 
and  long  periods  of  rest  observed.  The  food  should  be  nourishing  and 
sometimes  the  limitation  of  an  excessive  meat  diet  to  a  moderate  amount 
is  of  value.  In  any  event  alcohol,  coffee  and  spices  are  forbidden.  Mov- 
able kidneys  are  found  in  girls  even  at  puberty  possibly  from  wearing 
constricting  clothes  (Rosenthal,  Hollederer).  The  majority  of  cases  of 
Addison's  disease  observed  in  cliildhood  occur  at  puberty  corresponding 
to  the  common  appearance  of  tuberculosis  at  this  age  (Monti).  Ogston 
found  in  a  twelve  and  in  a  sixteen  year  old  girl,  besides  hypoplasia  of  the 
internal  genitals,  greatly  enlarged  suprarenal  glands.  As  far  as  the 
bladder  is  concerned  persistent  enuresis  generally  ends  at  puberty  with 
a  not  infrequent  increase  in  girls  up  to  the  beginning  of  menstruation. 
In  boys  this  annoyance  lasts  more  commonly  into  puberty  when  the 
prostate  gland,  w'hich  only  develops  to  a  considerable  extent  between 
the  tenth  and  fourteenth  years,  gradualh'  provides  sufficient  obstruc- 
tion during  sleep  (Dittel,  Bokai). 

Acute  infectious  diseases  come  with  the  greatest  frequency  in  ear- 
lier cliildhood;  according  to  our  observations  scarlet  fever  and  rheu- 
matism are  still  striking  at  the  beginning  of  puberty  on  account  of  the 
relatively  higher  morbidity.  Mumps  is  noteworthy  on  account  of  the 
inflammatory  metastases  of  the  sexual  glands  (testicles,  ovaries)  which 
not  infrequently  occur,  with  severe  local  symptoms.  As  regards  the 
chronic  infectious*  diseases,  the  great  frequency  of  tuberculosis  at  the 
period  of  puberty  (page  119)  has  already  been  considered.  Syphihs  is 
infrequently  observed  either  as  a  fresh  infection  following  premature 
sexual  intercourse  or  criminal  abuse,  or  as  late  hereditary  syphihs  with 
wliich  the  symptoms  of  the  inherited  dyscrasia  are  not  always  observa- 
ble in  earlier  cliildhood.  Of  the  manifold  e\idences  of  this  are  here  to 
be  mentioned:  hyperplastic  periostitis  of  the  tibia  or  of  the  head,  ster- 
num, etc.,  gummatous  processes  and  further,  obstinate  chronic  sym- 
metrical joint  affections  (suggesting  deforming  arthritis),  destructive 
processes  of  the  nasal  cartilage,  of  the  soft  parts  of  the  lung  and  of  the 


DISEASES    OF    PUBERTY  125 

pharynx  with  consequent  star-shaped  cicatrices,  indolent  glandular 
infiltration,  Hutchinson's  triad,  from  the  side  of  the  nervous  system  in 
addition  to  contractions  and  paralyses,  lesions  of  the  cranial  nerves,  also 
chronic  enlargement  of  the  liver  and  spleen  generally  without  icterus 
or  marked  ascites  and  finally  a  retardation  of  the  general  and  especially 
of  the  sexual  development  (Heubner,  Hoclisinger). 

Of  skin  affections  those  that  stand  in  close  connection  with  an  in- 
creased activity  of  the  sebaceous  glands,  comedones,  acne  and  furuncles 
are  to  be  mentioned  as  common  occurrences  in  the  years  of  puberty. 

Affections  of  the  eyes  with  the  exception  of  the  great  frequency  of 
myopia  at  the  time  of  rapid  growth,  are  not  infrequent  and  are  to  be 
considered  in  connection  with  the  hyperaemia  of  the  retina,  optic  nerve 
and  brain.  Acne  of  the  edge  of  the  Uds  (hordeolum)  is  often  seen  in  those 
at  puberty.  To  be  mentioned  are  the  pre-menstrual  retinal  hypersemias 
with  obscured  vision  and  headache,  disturbances  in  the  chorioid  mem- 
brane and  retina,  which  consist  of  exudation  and  haemorrhage,  but 
these  generally  go  on  to  complete  recovery.  Further  to  be  mentioned 
are  oedema  of  the  head  of  the  optic  nerve  with  immediate  improvement 
after  the  beginning  of  menstruation  and  a  periodic  return  before  each 
menstruation,  atrophy  of  the  optic  nerve  with  amenorrhoea  and  ful- 
minating tran,sitory  pre-menstrual  amaurosis  (Klopstock).  In  nervous 
individuals  concentric  contraction  of  the  field  of  ^ision,  asthenopia, 
hemcralopia  and  flittering  scotomata  are  seen;  sometimes  there  are 
luemorrhages  into  the  ^itreous  humour  wliich  recur  until  menstruation 
is  regularly  established.  Anaemic  girls  may  have  a  slight  inflammation 
of  the  whole  uveal  tract  (Gutman). 

As  has  been  mentioned  above  (see  page  115)  the  nervous  and  men- 
tal life  is  concerned  in  a  striking  manner  with  the  processes  character- 
izing puberty.  Children  who  have  been  previously  healthy  so  far  as 
their  nervous  system  is  concerned  not  infrequently  exhibit  phenomena 
at  tliis  time  wiiich  at  another  time  would  be  considered  pathological. 
A  congenital  neuropathic  disposition  may  now  first  become  noticeable 
or  irrational  hygienic  and  pedagogic  measures  at  home  and  in  school 
may  give  rise  to  considerable  nervous  disturbances.  First  and  foremost, 
headaches  are  a  common  occurrence  with  difi^ferent  locahzations,  some- 
times recurring  periodically,  sometimes  permanent,  so  that  the  ability 
to  pay  attention  and  to  perform  mental  work  may  be  very  greatly  af- 
fected. Active  hyperaemia  of  the  brain  is  not  infrequently  the  cause  of 
this;  this  phenomenon  is  often  associated  with  vertigo,  spots  before  the 
eyes  and  ringing  in  the  ears  and  possiblj'  also  hyperaemia  of  the  face. 
Passive  hyperaemia  often  results  from  wearing  clothes  wliich  compress 
the  neck  and  body,  from  bending  over  sharply  at  work  and  from  inter- 
ference with  nasal  breatliing.  Overstraining  of  the  eyes  on  account  of 
anomalies  of  refraction  is  to  be  considered.     Anaemia  of  the  brain  with 


1-26  THE   DISEASES    OF   CHILDREN 

anomalies  of  the  blood  and  insufficient  nourishment  can  give  rise  to 
headache  which  also  can  be  caused  by  mental  overstrain.  Other  fac- 
tors to  be  mentioned  and  which  are  also  to  be  observed  from  a  prophy- 
lactic standpoint  are  bad,  and  overheated  air  and  deficient  light  while 
at  work;  further,  dj'spepsia  and  constipation  and  finally  also  alcohol 
and  nicotine.  The  prophylaxis  and  treatment  will  be  found  under  the 
general  hygienic  and  tlictetic  measures  for  puberty  on  page  128.  Hemi- 
crania  not  infrecjuently  appears  at  this  time  if  it  has  not  previously 
existed,  in  which  case  it  may  cease.  Premonitory  symptoms  not  infre- 
quently precede  the  typical  attacks  of  headache  in  the  form  of  weak- 
ness, vertigo,  depression  and  irritabilit\';  vomiting  generally  follows  at 
the  height  of  the  attack  and  at  the  end  quiet  sleep  comes  on.  Over- 
filhng  the  stomach  often  acts  as  a  cause  as  does  the  ingestion  of 
alcohol,  emotions  and  physical  strain.  We  not  infrequently  observe  at 
puberty  the  symptoms  of  general  nervousness  based  upon  an  inher- 
ited tendency  and  this  shows  itself  in  the  form  of  a  permanent  excita- 
bility with  extraordinary  sensitiveness  to  mental  impressions  and  with 
explosive  reactions;  on  the  other  hand  we  frequently  meet  the  ready 
exhaustion  characteristic  of  neurasthenia,  caused  by  sUght  or  moderate 
mental  or  bodily  acti^'ity  in  which  sensations  of  pressure  in  the  head, 
languor,  pain  in  the  back  and  sleeplessness  may  appear.  Especially  in 
the  years  of  puberty,  epilepsy,  which  up  to  this  time  not  infrequently  has 
e\'idenced  itself  by  the  phenomena  of  temporary  loss  of  consciousness, 
or  in  attacks  of  vertigo,  reaches  full  development.  The  expectation 
often  raised  by  the  laity,  that  epileptic  convulsions  already  developed 
may  disappear  at  the  beginning  of  puberty,  is  unfortunately  seldom 
fulfilled;  rather  more  commonly  there  is  an  aggravation  of  them. 
While  the  greatest  frequency  of  chorea,  especially  the  so-called  rheumatic 
chorea,  occurs  in  the  school  j^ears  before  the  time  of  puberty,  chorea, 
especially  in  girls,  can  have  its  beginning  at  this  time.  In  addition  to 
the  characteristic  involuntary  movements  a  psychical  alteration  is  often 
very  striking,  in  the  form  of  irritability,  absent-mindedness,  weakness 
of  memory  and  slight  mental  exhaustion.  As  causal  factors,  emotions 
(fright  and  anxiety)  come  into  consideration.  The  chorea  which  on 
the  basis  of  imitation  breaks  out  occasionally  in  schools  almost  epidem- 
ically, belongs  in  the  domain  of  hj-steria.  The  congenital  diseased  ten- 
dency underlying  tliis  expresses  itself  in  early  childhood  generally  in 
single  local  symptoms  of  bodily  functional  disturbance  such  as  hypo-  or 
hyperkinesia.  Accidents,  emotions  and  psychical  contagion  are  effective 
causes.  With  progressive  bodily  development  a  childish  hysteria  may 
disappear  or  show  the  characteristic  protean  and  contradictory  form 
of  disease  in  wliich  purely  psycliical  conditions  may  with  extraordinary 
rapidity  take  on  the  most  various  physical  symptoms  of  disproportionate 
dimensions  and  duration.    In  addition  to  the  typical  convulsions,  attacks 


DISEASES    OF    PUBERTY  127 

may  appear  siniulatiug  unconsciuusiiess,  later  stupor,  prolonged  sleep 
and  somnambulism,  but  tliis  condition  never  goes  on  to  dementia. 

The  appearance  of  conspicuous  mental  weakness  is  an  earW  symp- 
tom of  hebephrenia;  tliis  stands  in  close  connection  with  the  rapid 
and  total  change  of  the  bodily  and  mental  development  and  always 
appears  in  connection  uith  puberty  (Hecker,  Kahlbauni).  Generally  the 
patients  are  indi\'iduals  already  backward  in  their  bodily  and  mental 
development  in  consequence  of  previous  illness,  in  whom,  often  appa- 
rently after  an  emotional  shock,  mental  depression  is  first  noticed  (with 
delusions  and  impression  of  persecution);  then  excitement  alternates 
with  causeless  mirth  and  a  tendency  to  foolish  speaking,  in  speech  and 
in  writing  a  dehght  in  arbitrary  imagery  appears,  often  -with  a  striking 
tendency  to  take  up  a  subject  over  and  over  again;  a  sentimental  method 
of  expression,  a  forced  tone  full  of  meaningless  phrases  and  affected 
speech  are  typical  phenomena.  An  exaggerated  impulse  to  be  doing 
sometliing  is  made  exadeut  by  aimless  actions,  such  as  httering  things 
about.  These  patients  may  remain  for  a  long  time  at  the  mid-point  of 
mental  decay  and  during  tliis  time  periods  of  excitement  amounting  to 
delirium  may  come  on  in  con.sequence  of  outward  causes  such  as  men- 
struation or  hallucinations.  Generally  the  termination  in  a  state  of 
extreme  mental  weakness  follows  in  the  course  of  a  few  months  often 
with  destruction  of  the  mind.  If  on  the  one  hand  the  phenomena  of 
hebephrenia  in  the  initial  stages  are  often  merely  the  same  that  we 
meet  in  health}' individuals  as  transitory  appearances  at  puberty,  on  the 
other  hand,  nevertheless,  victims  of  dementia  prsecox  often  show  cer- 
tain pecuharities  much  earlier;  these  are  a  reticent,  sly  manner,  whim- 
sicalness,  unruhness,  irritabihty  and  moral  instabiht}*. 

In  addition  to  the  hebephrenic  form  of  dementia  prcecox  described 
above,  other  forms  may  begin  at  puberty  and  lead  to  marked  mental 
weakness;  the  catatonic  form  for  instance,  in  which,  after  an  initial 
stage  of  depression,  states  of  stupor  alternate  with  excitement,  and 
there  appear  characteristic  phenomena  of  motor  spasm  and  motor 
retardation;  then  the  form  similating  paranoia,  characterized  by  hallu- 
cinations and  illusions  as  well  as  by  rapid  mental  decay.  Other  psy- 
choses may  now  and  then  be  observed  at  the  time  of  puberty,  and  it 
may  be  worthy  of  note  that  paralytic  dementia  not  infrequently  has  its 
beginning  at  this  period,  attacking  both  sexes  with  about  equal  fre- 
quency. According  to  Alzheimer  the  majority  of  such  cases  may  be 
traced  to  syphilis,  the  later  manifestations  of  which  appear  about  this 
time.  The  disease  runs  its  course  with  manj-  epileptiform  and  apo]ilec- 
tiform  attacks,  with  choreic  disturbances  and  especially  with  paralytic 
phenomena,  and  ends  rapidly  in  complete  dementia.  During  the  course 
of  the  disease,  states  of  excitement  and  of  confusion  are  (but  rarely)  to 
be  observed  while  optic  atrophy  is  common. 


1^8  THE   DISEASES   OF   CHILDREN 

The  great  frequency  of  diverse  pathological  processes  is  indicated  by 
the  above  consideration;  but  it  must  also  be  remembered  that  the  ■physio- 
logical sexual  development  is  a  critical  period,  requiring  much  insight  and  con- 
sideration jrom  parents  and  teachers.  In  the  following  pages  will  be  given 
merely  the  principles  of  a  rational   hygiene  for  the  periods  of  puberty. 

So  far  as  nutrition  is  concerned,  the  rapidly   growing   children   at 
the  age  of  puberty  require  an  increased  quantity  of  food,  more  than  the 
amount  necessary  for  adults.     The  latter  have  only  to  preserve  their 
equilibrium  by  supplying  the  material  that  is  used  up;  the  corporal 
development  of  puberty,  on  the  other  hand,  a  great  acceleration  as  it 
is  over  the  growth  in  preceding  years,  which  is  shown  by  the  gain  of  10 
Gm.  instead  of  5  Gm.  daily,  calls  for  an  increased  supply  of  food.    Accord- 
ing to  Voit,  Uffelmann  and  Ohlmiillcr,  the  average  of  food  required  daily 
consists  of  70-90  Gm.  of  proteids,  20-50  Gm.  of  fats,  and  500-250  Gm.  of 
carbohydrates.     Even  though  the  capacity  of  the  digestive  apparatus 
at  this  time  does  not  place  any  restriction  upon  the  quantity  or  quality 
of  the  food-stuffs.     Yet  special  consideration  must  be  exercised  in  ref- 
erence to  the  composition  of  the  food.     Of  the  special  indications,  the 
body  weight  is  to  be  considered  more  than  the  age;   moreover,  abundant 
exercise  in  the  fresh  air  is  required  with  a  diet  that  is  mostly  vegetarian. 
This  fact  is  especially  to  be  considered  with  those  children  that  may 
have  previously  been  accustomed  to  an  abundant  supply  of  proteids 
and  fats,  while  for  poorly  nourished  individuals  a  diet  especially  rich  in 
fats  is  sometimes  advisable.    In  all  cases  such  animal  food-stuffs  as  are 
rich  in  easily  assimilated  proteids,  for  example,  milk,  eggs,  and  cheese, 
are  never  to  be  placed  too  much  in  the  background,  especially  in  the 
diet  of  girls  inchned  to  chlorosis.    "With  boys,  on  the  other  hand,  a  diet 
too  abundant  in   meats  shows,  at  certain  periods  unfavorable  effects 
upon  their  state  of  nervous  excitability.    To  avoid  this  excitability  it  is 
advised   that  both   sexes  should   abstain  from   sharp   and  stimulating 
articles  of  diet,   such   as   mustard,  large  quantities  of  vinegar,   strong 
coffee,  tea,  spices,  and  alcohohc  beverages  of  any  kind.     Tobacco,  of 
course,  belongs  also  to  the  group  of  forbidden  articles.     It  must  be  in- 
sisted upon  that  sufficient  time  be  allowed  for  meals,  especially  in  the 
case  of  school  children.    So  far  as  evacuation  of  the  bowels  is  concerned, 
children  at  puberty  are  of  course  to  be  trained  in  regular  habits,  if  such 
habits  have  not  been  acquired  at  the  proper  time.     A  rational  nutrition 
by  means  of  a  simple  j'ct  not  monotonous  diet  is  a  necessary  condition 
for  normal   development.    Important  for   the   attainment  of  the  last- 
named  end  is  the  exclusion  in  dress  of  all  articles  of  clothing  that  may 
interfere  with  the  function  of  the  respiratory,  circulatory  and  digestive 
organs,  such  as  narrow  collars,   belts,  corsets  and  skirt-strings.     The 
weight  of  the  clothes  is  to  be  evenly  distributed  upon  shoulders  and 
hips.    Both  the  skeleton,  that  is  not  yet  perfectly  firm,  and  the  muscles, 


DISEASES    OF    PUBERTY  129 

not  yet  completely  developed  in  strength,  react  very  readily  to  injuries 
of  any  sort,  with  the  production  of  such  deformities  as  we  have  described 
above  (page  120).  Attention  is  to  be  paid  to  correct  posture  while 
writing,  as  well  as  sufficient  light  during  work  in  the  school  or  at  home. 
Sitting  down  for  long  periods  of  time  occupied  with  work  requiring  men- 
tal effort  is  an  injurious  factor  of  first  importance  at  the  time  of  puberty; 
and  to  combat  this  the  temporary  freedom  from  all  work  is  often  more 
rational  than  additional  tasks  that  seem  to  suit  the  mental  progress  of 
the  child.  Increase  of  work  requiring  mental  effort  is  to  be  regulated 
in  this  period  solely  by  the  progress  of  bodily  development.  Rational 
diri.-^ion  of  time  between  work,  relaxation,  and  rest,  with  thorough  use 
of  the  periods  allotted  to  each,  is  very  important  in  each  case.  While 
in  general  almost  all  the  hours  at  school  are  devoted  to  the  mental  edu- 
cation, the  attention  to  the  physical  development  is  relegated  almost 
exclusively  to  the  parental  home,  where  private  studies  of  all  sorts  in 
addition  to  the  required  school  tasks  lead  to  overwork  of  the  brain, 
that  is  so  sensitive  during  this  period.  In  order  that  a  race  healthy  in 
body  and  nerves  may  be  raised,  it  is  necessary  to  make  changes  in  the 
above  arrangement.  The  school  must  assign  more  time  to  compulsory 
physical  exercise,  while  the  requirements  in  mental  work  are  to  be  cor- 
respondingly diminished,  yet  the  fact  must  not  be  lost  sight  of,  that 
systematic  physical  exercise  is  not  necessarily  equivalent  to  relaxation 
in  all  individuals.  The  various  sports  that  have  become  so  popular  at 
the  present  time  are  valuable  factors  in  opposing  the  injury  done  by 
the  school,  provided  they  are  regulated  according  to  individual  capaci- 
ties, and  are  not  allowed  to  obtain  complete  hold  of  the  sphere  of  men- 
tal interests.  Excessive  physical  exercise  can  easily  do  harm  in  the 
period  of  puberty.  While  systematic  gymnastic  exercise  strengthens 
the  muscles,  and  exercise  especially  in  the  fresh  air,  by  such  means  as 
rowing,  swimming,  cycling,  skating,  tennis  and  ball  games  produces 
good  effect  upon  the  capacity  of  the  thorax  by  the  increased  expan- 
sion of  the  lungs,  as  well  as  upon  the  various  groups  of  muscles  and 
the  general  bodily  agility,  yet  overfatigue  may  result  with  grave  sequels 
in  its  train.  Accordingly  the  duration  and  intensity  of  exercise  is  always 
to  be  carefully  limited;  this  is  especially  true  of  cychng,  which  like  other 
too  strenuous  exercises  easily  leads  to  overstraining  the  heart,  ready  as 
the  latter  is  to  break  down  at  this  period  of  development.  Too  prolonged 
a  stay  in  cold  water  is  especially  to  be  forbidden  to  growing  girls  inclined 
to  be  chlorotic.  Often  not  enough  importance  is  attached  to  the  hygienic 
value  of  a  daily  prolonged  walk  in  the  fresh  air.  The  rational  exercise 
of  the  body  leads  to  a  wholesome  fatigue,  which  is  made  up  for  in  the 
period  of  sound  sleep.  The  latter  is  an  especial  necessity  for  individuals 
at  the  time  of  puberty,  and  care  must  be  taken  that  its  duration  be 
perfectly  sufficient  and  extend  from  9  to  10  hours. 
II— 9 


130  THE    DISEASES   OF   CHILDREN 

If  we  remember  the  necessity  for  sufficient  sleep,  and  the  various 
injuries  that  may  be  prockiced  by  nervous  excitement,  sensual  stimula- 
tion, alcoholic  excesses  and  the  Uke,  it  becomes  clear  that  individuals 
at  the  age  of  puberty  are  best  prevented  from  taking  part  in  the  so- 
called  social  pleasures  of  any  sort,  unless,  of  course,  the  latter  are  made 
suitable,  from  every  standpoint,  to  the  stage  of  development  of  their 
age.  In  the  sphere  of  education  especial  consideration  is  to  Ix'  given  to 
the  nervous  and  mental  Ufe  of  such  individuals.  Individuahzed  lo^dng 
care  and  handHng  by  considerate  parents  and  teachers  lead  most  chil- 
dren more  easily  and  smoothly  through  this  critical  period,  than  the 
adoption  of  a  strict  routine,  although  at  times  energy  and  insistence 
ought  to  be  displayed.  It  is  important  to  keep  guard  over  the  reading 
matter,  the  amusements  and  the  social  intercourse  of  the  child,  while 
one  of  the  tasks  of  a  rational  education  nowadays  is  to  furnish  informa- 
tion in  regard  to  the  natural  sexual  processes.  This  may  best  be  done 
by  the  parents  themselves,  with  the  use  of  examples  taken  from  the  hfe 
of  plants  and  animals  (Siebert).  If  the  parents  or  teachers  of  the  child 
are  nervous  themselves,  very  grave  conflicts  may  occur  at  this  time. 
In  order  to  prevent  such  conflicts  and  to  remove  the  harm  that  may  be 
done  by  such  bad  example  to  the  already  sensitive  nervous  organism  of 
the  growing  child,  it  is  often  advisable,  from  the  physician's  standpoint, 
to  remove  children,  endangered  in  such  fashion,  from  the  school,  or  from 
their  home,  or  even  from  life  in  the  city,  unfavorable  as  the  latter  often 
is  in  other  conditions.  In  such  cases,  the  boarding  schools  in  the 
country  are  best  suited  to  guide  the  child  correctly  in  passing  through 
the  period  of  puberty. 


Constitutional  Diseases 


DISEASES  OF  THE  BLOOD  AND  OF  THE  BLOOD- 
PREPARING  ORGANS 

BY 

Dr.  a.  JAPHA,  of  Berlin 

translated   bv 
Dr.  EDWARD  F.  WOOD,  Wilmington,  N.  C. 


PRELIMINARY   REMARKS— PHYSIOLOGY 

The  microscopic  blood  picture  in  infantile  blood  diseases  becomes 
intelligible  only  through  knowledge  of  the  normal  blood  in  infancy. 

The  blood  of  the  newborn  infant  exhibits  the  following  peculiar- 
ities according  to  the  latest  pubhcations  of  Schiff,  Perlin,  Carstanjen, 
ScipiadeS;  and  Takasu: 

1.  A  very  high  specific  gravity,  1.060-1.080,  as  against  1.050- 
1.060  in  the  adult. 

2.  A  high  percentage  of  haemoglobin,  100  to  140  per  cent,  of  the 
percentage  in  the  healthy  adult. 

3.  A  specially  large  number  of  red  blood  corpuscles,  5,825,000- 
7,550,000. 

4.  An  increased  number  of  white  blood  cells,  up  to  36,000. 

5.  A  preponderance  of  polynuclear  cells,  73.4  per  cent,  polynuclear, 
16.05  per  cent,  lymphocytes  on  the  first  day  (Carstanjen). 

6.  Nucleated  red  Ijlood  corpuscles  (as  in  the  placental  blood,  where 
they  are  more  numerous),  up  to  the  third  day;  up  to  the  sixth  day  only 
in  very  few  cases  (Takasu),  afterwards  hardly  any. 

7.  All  these  differences  are  considerable  up  to  the  fourth  day, 
then  they  grow  less;  at  the  middle  or  end  of  the  first  montli  both  haemo- 
globin and  number  of  red  blood  corpuscles  have  arrived  at  about  the 
level  of  the  adult  man;  the  number  of  polynuclear  leucocytes  dimin- 
ishes like^\^se  and  the  percentage  of  the  various  kinds  of  leucocytes 
from  the  fourth  day  is  about  the  same  as  it  will  be  during  the  first 
few  months. 

No  doubt  some  of  the  blood  changes  in  the  newborn  are  caused  by 
the  lack  of  water,  considerable  passing  out  through  the  intestines  and 
perspiration,  and  in  consequence  of  small  supply. 

131 


132 


THE   DISEASES   OF   CHILDREN 


In  infancy  there  are  also  altered  blood  conditions  as  compared  to 
the  adult  (Fig.  20,  and  Fig.  27,  page  166),  as  follows: 

1.  Decreased  amount  of  haemoglobin  down  to  10.35  Gm.,  in  the 
average   11.5  Gm.  (as  against  13-14  Gm.  in  the  adult). 

2.  Often  slight  decrease  of  the  red  blood  corpuscles. 

3.  Increase  of  the  white  blood  cells;  about  12,000  to  13,000. 

4.  Preponderance  of  lymphocytes:  about  50-55  per  cent.  The 
lymphocytes  of  infancy  show  greater  differences  in  size  than  in  the 
adult;  there  are  also  larger  sizes.  (These  lymphocytes  originate  accord- 
ing  to   Ehrlich    exclusively   from   the  lymph-nodes;   but   according   to 

Fin.  20. 

Curve  of  liEpmoglobin  ( "),  of  the  red  ( 1  and  colorless  ( )  blood  corpuscles 

at  various  periods  of  age. 
Children  (Largely  after  Schwinge,  Preisschrift  Goettingen.  1898) 

Men 


Women 


r.  I.,  c. 
5500000 
5400000 
5300000 
5200000 
5 1001 II  Ml 
500001 W I 
4900000 
4800000 
4700000 
4600001) 
4500000 
4400000 


iMlBHBBHg 


"        o       o       o       o 

»0  O  t--  00 

i   4   i   i 


l,b. 

w.  b.  c. 

hb. 

19.5 

13000 

19.5 

18.9 

12250 

18.9 

18.3 

11500 

18.3 

17.7 

10750 

17.7 

17.1 

10000 

17.1 

111.5 

9250 

10.0 

l.-,.9 

8500 

15.9 

15.3 

7750 

15.3 

14.7 

7000 

14.7 

14.1 

0250 

14.1 

13.5 

5500 

13.5 

12.9 

4750 

12.3 

The  cur\-es  show  the  exceedingl.v  large  quantities  of  htemoglobin.  red  and  colorless  blood  corpuscles 
shortly  after  birth,  and  the  decrease  after  the  first  few  days  of  life.  The  leucocyte  cur\'e  remains  almost 
during  the  entire  first  year  above  the  height  of  later  life,  whereas  the  hEemoglobin  and  red  corpuscles  sink 
below  and  only  gradually  ascend  again,  .\fter  the  fifteenth  year  the  quantity  of  haemoglobin  and  white  blood 
corpuscles  increases  in  tlie  male  sex  and  remains  higher  than  in  the  female  throughout  life. 

Grawitz,   Walz  and  Pappenheim,  similar  cells  exist  also  in  the  bone 
marrow.) 

5.  According  to  some  investigators  (Carstanjen,  Karnizki)  the 
so-called  transitional  forms  are  increased  to  about  8  or  10  per  cent,  (in 
the  adult  there  are,  together  with  the  large  mononuclear  cells,  only 
2-4  per  cent.,  according  to  Ehrlich-Lazarus). 

6.  Myelocytes  (mononuclear  cells  with  neutrophile  granulation) 
are  sometimes  found  in  the  healthy  infant  during  the  first  few  weeks  of 
life,  but  always  in  sUght  numbers,  never  later  (Zelenski-Cybulski). 

7.  Normoblasts  are  met  with  but  rarely  (Geissler  and  Japha, 
Zelenski-Cybulski,  Karnizki),  although  formerly  their  occurrence  even 
in  healthy  children  was  considered  the  rule. 


DISEASES   OF   THE   BLOOD  13S 

From  the  second  year  the  blood  gradually  approaches  the  normal 
blood  of  the  adult,  a  state  which  is  not  attained,  however,  until  the  fif- 
teenth or  twentieth  year.  Up  to  the  third  to  sixth  year  there  is  a  pre- 
ponderance of  lymphocytes.  Sex  does  not  cause  any  material  difference 
in  the  blood  picture  up  to  the  fifteenth  year. 

Japha  has  not  been  able  to  demonstrate  a  proper  leucocytosis  of 
digestion  in  the  bottle-fed  infant,  Gregor  did  not  even  find  it  regularly 
in  the  breast-fed  infant.  Moro  fountl  a  decrease  of  leucocytes  one-half 
to  two  hours  after  ingestion  of  food  and  seems  to  regard  this  leukopenia 
as  a  regular  occurrence.  If,  however,  a  breast-fed  infant  was  given 
cow's  milk  (heterogeneous  albumin),  there  was  an  immediate  occurrence 
of  leucocytosis.  Unfortunately  the  time  of  day  when  these  experiments 
were  made,  is  not  stated.  Besides,  leucocyto.sis  of  digestion  does  not 
seem  to  be  quite  regular  even  in  the  adult,  and  the  curve  of  leucocytes 
changes   also   during   the   day. 

The  blood-making  organs  of  the  infant  are  severely  affected  by  dis- 
ease. The  infantile  blood  easily  takes  up  myelocytes  and  nucleated  red 
blood  corpuscles  (Zelenski-Cybulski).  In  infectious  diseases  the  infant 
is  perfectly  capable  of  producing  polynuclear  leucocytosis.  Measles  do 
not  produce  leucocytosis  (Felsenthal,  Plantenga,  Caccia,  Tschisto witch 
and  Schestakof).  In  whooping-cough  hyperleucocytosis,  with  particular 
increase  of  lymphocytes  was  found  even  in  afebrile  cases  (up  to  47,000, 
de  Amici  and  Pauhoni,  Cima).  In  severe  cases  of  diphtheria  Engel 
found  3.6-16.4  per  cent,  myelocytes.  In  intestinal  affections  of  infants 
Schlesinger  and  others  found  lymphocytosis,  Japha  however  was  unable 
to  confirm  this. 

SYMPTOMATIC  ANEMIAS  INCLUDING  SCHOOL  AN.ffi;MIA 

Symptomatic  anaemias  deserve  a  separate  discussion  inasmuch  as 
they  depend  so  greatly  upon  their  causes,  and  in  many  cases  the  recog- 
nition of  the  etiology  (hunger,  for  instance)  is  equivalent  to  a  cure.  The 
blood  picture  is  a  resultant  of  the  injurious  influences  acting  upon  the 
blood-forming  organs  and  the  reactive  capacity  of  these  organs:  the 
weaker  these  organs,  the  more  readily  will  anaemia  be  produced. 

Anaemia  after  Haemorrhage. ^The  cause  of  aniTmia  most  easily 
recognized  is  haemorrhage  (from  an  umbilical  wound,  in  consequence  of 
melsena,  infantile  scurvy,  epistaxis,  ha-morrhagic  diathesis,  intestinal 
haemorrhage,  polypi,  intussusception,  gastric  ulcer,  intestinal  parasites). 
If  the  acute  losses  of  blood  do  not  cause  death,  the  infant  will  undergo 
rapid  repair.  Sometimes  severe  ha^norrhage  is  not  followed  by  uninter- 
rupted recovery,  but  may  result  in  a  prolonged  and  deleterious  anirmia. 

The  therapy  of  acute  anamiia  requires,  after  checking  the  haemor- 
rhage, hypodermatic  and  intestinal  saline  infusions,  heat,  autotransfu- 
sion  by  lowering  the  position  of  the  head  and  wrapping  up  the  extremi- 


134  THE    DISEASES   OF   CHILDREN 

ties,  and  finally  administering  nourishing,  but  easily  digestible  food. 
Iron  therapy  is  not  very  important  in  acute  cases  of  loss  of  blood,  be- 
cause the  latter  in  itself  incites  the  blood-forming  organs  to  renewed 
activity,  and  the  ordinary  food  should  as  a  rule  contain  sufficient  iron. 
Infants,  however,  should  receive  small  doses  of  iron  because  milk  is 
deficient  in  iron.  Suitable  preparations  are  ferrum  oxyd.  solub.  (P.  G.) 
0.03-0.05  Gm.  (4-1  grain)  three  times  daily,  ferrum  lactic.  (P.  G.)  0.01 
Gm.  (g  grain)  three  times  daily,  hqu.  ferri  albuminat.  (P.  G.)  8-10 
drops  three  times  daily;  it  is  important,  however,  that  the  organs  of 
digestion  be  in  good  condition. 

ANiEMIA  AFTER  ILLNESS  (INCLUDING  POISONING) 

Another  cause  of  ana?mia  may  be  disease  processes  and  poisons. 
Poisoning  by  lead,  arsenic,  and  mercury,  is  not  of  frequent  occurrence  in 
infants,  but  poisoning  may  happen  through  drugs  in  the  administration 
of  guaiacol,  extract  of  male  fern,  potassium  chlorate,  pA^-ogallic  acid, 
phenacetin,  phenocoll,  lactophenin.  These  substances  either  dissolve 
the  red  blood  corpuscles  or  change  the  haemoglobin  into  an  inactive 
modification  (e.g.,  methamoglobin).  Of  pathological  conditions,  the 
follomng,  aside  from  those  which  lead  to  hemorrhage:  ha?moglobinuria, 
malaria,  albuminuria,  dysentery,  other  intestinal  affections,  infantile 
scurvy,  sepsis  and  intestinal  parasites  (ankylostomum,  bothrioceph- 
alus,  trichocephalus,  also  ascaris,  according  to  Demme).  Congenital 
syphilis  may  be  responsible  for  particularly  severe  ansemia  (Loos),  but 
not  all  cases  of  sypliilis  are  ansemic,  nor  do  they  all  become  so  during 
observation. 

The  many  causes  of  anajmia  make  it  incumbent  upon  the  physician 
not  to  be  satisfied  with  the  diagnosis  alone  of  ana-mia,  but  to  search  for 
the  cau.se.  This  includes  a  thorough  physical  examination;  Ukemse  an 
examination  for  intestinal  parasites,  which  should  never  be  neglected. 
In  severe  cases  after  purgative  medication  several  stools  should  be 
thoroughly  examined  microscopically.  Symptoms,  course,  and  treat- 
ment of  this  form  of  chronic  anaemia  depend  upon  the  cause. 

ANvEmA  IN  CONSEQUENCE  OF  M.ALNUTRITIOX,  ETC. 

Deficient  or  faulty  nutrition  is  in  infants  a  further  cause  of  symp- 
tomatic anaemia;  the  somewhat  complicated  conditions  are  well  described 
by  Panum,  Voit,  Senator,  Fr.  Miiller,  Kieseritzky,  and  von  Hos-shn. 
Absence  of  iron  in  the  food  seems  to  have  a  particularly  injurious  effect 
(Ehrlich-Lazarus).  The  severest  results  are  probably  produced  during 
the  period  of  active  bone-formation  toward  the  end  of  infancy  and  dur- 
ing puberty.  This  also  explains  the  anaemia  of  infants  who  have  been 
fed  too  long  on  an  exclusive  milk  diet.  According  to  Bunge  the  new- 
born possess  a  comparatively  large  amount  of  iron;  nulk,  however,  is 


DISEASES   OF   THE    BLOOD  135 

very  poor  in  iron,  so  that  the  amount  of  iron  stored  up  will  only  last  for 
a  certain  time,  after  which  the  deficiency  of  iron  xNdll  make  itself  felt. 
This  form  of  anaemia  cannot  be  cured  by  medication;  the  appe- 
tite should  be  stimulated  and  the  necessary  directions  given  for  proper 
nutrition.  Poor  parents  should  be  shown  how  the  necessary  quantity 
of  calories,  proteids  and  iron  can  be  furnished  in  the  cheapest  way; 
to  the  rich  it  should  be  explained  that  lean  meat  alone  is  not  sufficient 
as  food,  that  an  excess  of  proteids  increases  the  internal  work  of  the 
organism,  that  fat  is  by  no  means  as  injurious  as  is  frequently  supposed, 
that  milk-fat  (butter)  can  even  be  borne  well,  and  that  finally  fresh  veg- 
etables and  potatoes  are  very  desirable  food  for  children.  The  following 
table  by  Bunge  shows  the  percentage  of  iron  contained  in  a  number 
of  different  food-stuffs: 

100  Gr.uis  of  Dry  Substance  Coxtaix  Milligrviis  of  Iron  : 

White  of  eggs Trace         Peas 6 . 2-6 . 6 

Maize 1 . 0-2 . 0     Cherries,  black,  without  stones  .  .   7.2 


Peeled  barley 1 . 4-1 . 5 

Wheat  flour 1.6 

Cow's  milk -. .  .   2.3 

Woman's  milk 2.3-3.1 


Beans,  white 8.3 

Carols 8.6 

Strawberries 9.3 

Lentils 9.5 


Figs 3.7  I  Almonds,  brown  skin, 9.5 

Raspberries 3.9     Cherries,  red,  without  stones  ....    10.0 

Peeled  hazel  nuts 4.3      Hazel  nuts,  brown  skins 1.3 .0 

Barley 4  .5     Apples 13.0 

Cabbage,  inner  yellow  leaves 4  5     Cabbage,  outer  green  leaves 17.0 

Peeled  almonds 4  9     Asparagus 20 . 0 

Rye 4.9 

Wheat 5.5 

Whortle  berries 5.7 

Potatoes 6  4 


Yolk  of  egg 10.0-24.0 

Spinach 33.0-39.0 

Pig's  blood 226.0 

Haemoglobin 240 . 0 


ililk  and  fine  flour,  and  therefore  also  wliite  bread,  belong  to  the 
foods  poorest  in  iron,  w'hereas  the  opposite  is  true  of  spinach,  asparagus, 
apples,  cherries;  also  potatoes,  peas  and  beans  contain  a  fair  propor- 
tion of  iron.  For  this  reason  infants  should  not  be  fed  too  long  on  milk, 
not  even  mother's  milk. 

The  presumably  unfavorable  influence  of  insufficient  fight  and 
vicious  air  could  not  be  proved  l?}'  experimentation  (Schonenberger, 
Fliigge).  It  is  possible,  however,  that  in  nervous  children  and  those 
with  a  weak  constitution,  especially  infants,  tliis  may  be  different.  The  e^^l 
effects  of  overheating,  however,  are  well  estabfished  (Fliigge,  Grawitz). 

AN.EMIA  OF  SCHOOL  CHILDREN 

Various  unfavorable  influences  are  at  work  in  the  development  of 
school  anaemia.  Vitiated  air  may  cause  a  bad  effect  by  acting  on  the 
nervous  sj^stem  and  thus  causing  disgust  in  tender  children;  overheating 
is  also  a  factor,  especially  if  the  heat  is  suppfied  from  a  central  finnace; 


136  THE   DISEASES   OF   CHILDREN 

further,  exertion,  purely  psychic  causes,  longing  for  the  mother,  ambition, 
anxiety;  finally  the  lack  of  fresh  air  may  well  be  of  greater  importance 
than  the  presence  of  bad  air.  Some  children  become  affected  in  the  first 
six  months  of  their  school  work;  others  only  in  later  years,  as  increased 
studies  make  greater  demands  upon  them.  The  children  of  the  poor 
are  distinctly  more  affected  in  large  cities  than  those  of  the  rich.  This 
is  even  more  so  in  children  of  widows,  which  is  explained  not  only  by 
unfavorable  surroundings,  but  also  by  unsuitable  psychic  influences  on 
the  part  of  the  mother.  Here,  as  in  all  forms  of  anaemia,  hereditary 
tendency  and  especially  nervous  debility  play  a  considerable  role;  ill- 
ness or  advanced  age  of  the  parents  at  the  time  of  birth  of  the  children, 
tendency  to  migraine  and  similar  disturbances,  favor  the  development 
of  school  anaemia  and  make  its  cure  more  diflficult. 

Symptoms. — The  subjective  complaints  and  nervous  troubles  are 
fatigue,  heaviness  in  the  legs,  headaches  which  occur  principally  in 
school,  vomiting  at  school  which  in  severe  cases  may  be  repeated  almost 
daily.  Especially  after  bodily  exertion,  such  as  gymnastic  exercises, 
there  is  pain  in  the  sides  and  epigastric  region.  Parents  notice  the  de- 
pressed condition  of  the  child,  the  decreased  vitality,  increased  desire 
to  sleep,  and  diininished  incUnation  to  take  part  in  games.  Sleep  is 
restless  and  often  disturbed  by  attacks  of  pavor  nocturnus.  There  is 
less  appetite  (the  lunch  is  brought  back  home),  and  the  bowels  are 
irregular. 

The  objective  signs  are:  weakened  constitution,  pallor  of  the  form- 
erly fresh  complexion  and  mucous  membranes,  decrease  of  the  subcuta- 
neous fat  and  a  striking  flabbiness  of  the  muscles.  Respiration  and  pulse 
are  frequently  accelerated,  the  pulse  may  be  full,  but  soft.  Examination 
of  the  blood  shows  a  decrease  of  haemoglobin  and  of  red  blood  corpus- 
cles; sometimes  the  latter  show  differences  of  form,  also  a  slight  in- 
crease in  leucocytes;  usually  the  changes  are  not  very  pronounced  or 
they  may  be  absent  altogether  in  spite  of  severe  clinical  symptoms. 

Cardiac  dulness  is  sometimes  slightly  increased;  the  impulse  may 
be  widened  and  surprisingly  strong.  Heart  murmurs  are  found,  espe- 
cially after  slight  exertions,  and  the  first  sound  may  he.  reduplicated  or 
impure,  or  accompanied  by  a  slight  murmur.  Over  the  cei'vical  veins 
a  venous  hum  may  be  distinguished  even  in  infants. 

Many  otherwise  robust  children,  who  are  at  once  affected  in  their 
first  school  year  by  the  mere  change  of  conditions,  or  perhaps  later 
owing  to  unfavorable  exterior  influences,  recover  rapidly  after  a  short 
vacation;  others,  if  once  affected,  may  drag  the  trouble  with  them 
throughout  their  school  life.  These  are  usually  weak  children  with  an 
hereditary  taint.  In  cases  of  this  kind  the  prognosis  is  doubtful  in  view 
of  the  tendency  of  the  debihty  to  persist  until  a  later  period  of  Hfe. 
These  children  are  also  apt  to  develop  scrofula. 


DISEASES    OF   THE    BLOOD  137 

The  diagnosis  requires  careful  exclusion  of  other  affecti-ons  (scrof- 
ulosis,  tuberculosis,  intestinal  parasites). 

Therapy. — The  necessity  of  removing  the  original  cause  would 
make  it  desirable  to  keep  the  children  away  from  school  for  a  time. 
In  serious  cases  this  should  always  be  done,  as  the  injurious  influences 
which  act  on  both  body  and  mind  are  thereby  counteracted.  In  the 
first  stages  of  the  trouble  a  few  weeks'  rest  is  frequently  sufficient,  but 
in  a  case  of  long  standing  prolonged  rest  is  required.  After  recovery 
has  taken  place,  it  is  generally  easy  for  the  refreshed  brain  to  make  up 
for  lost  time.  In  obstinate  cases  where  the  school  again  and  again  causes 
the  trouble  to  break  out  afresh,  a  cure  .should  be  attempted  in  spite  of 
the  child  attending  school.  Well-to-do  famihes  may  for  a  time  have 
recourse  to  private  lessons.  For  the  poorer  part  of  the  population  schools 
situated  in  the  woods  are  excellent  in  this  respect.  It  is  important  to 
correct  mistakes  of  education  at  home,  to  tone  down  the  ambition  of 
the  mother  in  wishing  her  chiUl  always  to  be  the  first,  to  omit  the  mu.sic 
lesson,  etc.  Among  the  poor  too  much  house  work  (see  the  Care  of  Chil- 
dren in  Poor  Famihes);  or  using  children  for  business  purposes  such  as 
delivering  newspapers,  are  also  great  exertions  and  shorten  the  hours  of 
sleep.  The  time  out  of  school  should  be  largely  devoted  to  .sleep,  also 
during  the  day  a  few  hours  of  sleep  should  be  inserted,  perhaps  immedi- 
ately after  school  or  before  dinner.  Children  should  be  in  the  fresh  air 
as  much  as  possible.  The  arrangements  which  permit  children,  includ- 
ing the  poor,  to  spend  at  least  a  few  weeks  of  the  year  in  really  good  air, 
are  to  be  hailed  with  dehght.  Places  of  recreation,  vacation  colonies, 
sea  resorts,  summer  colonies  and  rural  sanitaria,  or  pos.sibly  a  visit  with 
relatives  in  the  country,  are  desirable.  Even  if  after  return  to  town  the 
body  weight  and  percentage  of  hiemoglobin  should  fall  again,  in  the 
majority  of  cases  an  improvement  still  remains  as  compared  to  the  con- 
dition before  the  commencement  of  the  cure.  The  children  of  well-to-do 
parents  are  sent  to  rural  sanitaria  or  summer  resorts.  The  best  places 
are  those  where  the  climate  is  warm  and  yet  affords  sufficient  protection 
from  excessive  heat.  In  the  country,  heat  is  much  more  bearable  than 
in  the  streets  of  great  cities,  whore  the  gigantic  piles  of  stone  and  mortar 
absorl)  the  heat  of  the  sun  and  do  not  easily  become  cool  again.  An;r- 
mic  children  can  bear  heat  better  than  cold,  and  summer  resorts  in  the 
flat  country  are  very  suitable,  also  watering  places  (there  is  no  necessity 
for  taking  the  baths),  whereas  many  places  in  wooded  districts  have 
too  many  cold  and  wet  days.  The  climate  near  the  sea  has  an  evil  rep- 
utation in  regard  to  its  effect  upon  aniBuiic  patients.  Those  endowed 
with  a  fairly  good  power  of  resistance  may  be  sent  to  the  seaside,  but 
care  should  be  taken  not  to  expose  them  to  the  hot  rays  of  the  sun 
and  not  to  allow  them  to  bathe  in  the  sea.  With  many  young  children 
the  climate  at  high  altitudes  agrees  well,  which  in  the  opinion  of  very 


138  THE   DISEASES   OF   CHILDREN 

competent  physicians  may  have  a  salutary  effect  in  very  obstinate  cases. 
Very  high  altitudes,  however,  are  not  suitable  for  every  child.  High 
altitudes  i)robably  act  less  by  the  increase  of  red  blood  corpuscles,  than 
by  improved  metaboUsm  (Miescher,  Loewy  and  Zuntz,  Schumburg  and 
Zuntz,  Meissen  and  Schroeder,  Gottstein).  As  to  the  use  of  chalybeate 
waters,  which  may  also  be  used  in  the  case  of  young  children,  more  will 
be  said  in  a  subsequent  paragraph. 

Hardening  of  the  body  is  only  justified  where  overanxious  parents 
pamper  the  child;  otherwise  anirmic  children  require  indulgent  care. 
They  should  not  be  persuaded  to  take  long  walks  or  to  pla)'  games  which 
require  much  exercise.  Gymnastic  exercises,  which  frequently  cause 
trouble,  should  he  discarded.  After  the  requisite  degree  of  strength  has 
been  gained,  which  is  well  indicated  by  the  gain  in  body  weight,  it  is 
advisable  to  take  brisk  walks  even  in  cold  winter  weather,  and  to  warm 
the  hands  and  feet  by  brisk  movements  at  play.  Then  also  gymnastic 
exercises  are  indicated.  Smmming  or  cold  frictions  frequently  exacer- 
bate the  trouble  considerably,  because  the  child  is  not  nourished  well 
enough  to  stand  the  loss  of  body  heat,  so  that  no  reaction  of  the  vessels 
takes  place.  In  these  cases  it  is  better  to  prescribe  friction  with  coarse 
towels  which  are  gradually  and  carefully  replaced  by  rubbing  with  eau 
de  Cologne  or  brandy  and  finally  by  a  wet  sheet.  The  child  should  be 
rubbed  immediately  on  being  taken  out  of  the  bed,  and  afterwards  be 
placed  back  into  bed  for  a  few  minutes.  Rubbing  may  also  take  place 
after  a  warm  bath,  but  the  chief  concern  is  that  the  child  invariably  be 
warm  after  the  treatment.  Later  on  it  is  well  to  let  the  children  splash 
about  with  their  feet  in  cold  water.  Many  authors  recommend  sweat 
baths  in  the  case  of  very  young  chlorotic  children. 

Great  attention  should  be  paid  to  sufficient  nutrition.  Five  meals 
should  be  kept  up.  Before  getting  up  in  the  morning  and  in  the  evening 
some  milk  may  be  given  to  the  child  in  bed.  Before  going  to  school  the 
child  should  partake  of  a  proper  meal  containing  plenty  of  albumin 
(milk,  buttered  bread,  and  if  possible,  egg  or  meat).  Then  also  the 
second  breakfast  will  usually  be  eaten,  and  in  many  schools  provision 
is  made  for  allowing  the  children  a  glass  of  good  cool  milk.  Before 
dinner  rest  is  desirable.  Generally  .speaking,  the  child  may  eat  what- 
ever it  likes,  so  long  as  it  eats. 

Personal  taste  may  be  gratified  by  allomng  caviar,  small  quanti- 
ties of  sardines,  ginger,  pickled  melon;  older  children  may  have  a  piece 
of  herring,  sardines,  cucumbers,  salads  which  may  be  dressed  with  lemon 
(beans,  tomatoes,  celery,  asparagus,  endives,  green  salads;  in  dressing 
leaf  salads  great  cleanhriess  should  be  observed).  By  these  dishes  and 
plenty  of  sauces,  and  by  coating  meat  dishes  with  fruit  jams,  meat  can 
be  more  easily  administered.  In  the  case  of  younger  children  who  can- 
not masticate  well,  the  meat  should  be  cut  into  very  small  pieces  or 


DISEASES   OF   THE    BLOOD  139 

mashed.  For  this  purpose  meat  cutting  machines,  scissors,  mastica- 
tors, etc.,  have  been  invented.  Food  containing  a  high  percentage  of 
iron  is  preferable  (see  Table  on  p.  135).  Vegetables  can  be  cooked  very 
nicely  with  butter,  cream  is  also  well  borne,  especially  mth  strawberries 
(caution  on  account  of  decomposition  after  standing);  many  children 
are  fond  of  Unseed  oil  (mth  potatoes).  For  "fattening"  may  be  recom- 
mended, oatmeal,  red  grits,  jelUes;  honey  is  very  nutritious  (1  table- 
spoonful  equal  to  75  calories).  Older  children  are  given  for  a  few  weeks 
(only  to  stimulate  the  appetite)  a  little  wine  (sweetened  claret,  a  liquor 
glass  to  half  a  -nineglassful  t\\ice  a  day),  a  glass  of  malt  beer  (2-3  per 
cent,  alcohol,  7.5-10  per  cent,  malt  extract). 

Proprietary  foods  should  only  be  given  if  the  ordinary  food  cannot 
be  administered  in  sufficient  quantities.  Only  few  fat  preparations  are 
suitable  for  continued  use;  oil  of  sesamum  and  codliver  oil  (twice  daily 
a  teaspoonful  to  a  tablespoonful),  the  latter  also  in  the  form  of  os.-5in 
stroschein  (a  combination  with  the  egg  albumin,  in  tablespoonful 
doses)  also  effervescent  oil  are  generally  well  liked.  Moreover,  chocolate 
or  cocoa  containing  oleic  acid  is  well  assimilated  according  to  Zuntz, 
and  finally  lipanin  (olive  oil  ^\ith  6  per  cent,  oleic  acid,  in  teaspoonful  to 
desertspoonful  doses)  though  this  is  expensive.  Of  carbohydrates  may 
be  mentioned:  oats-cocoa,  Theinhardt's  hygiama,  malt  extract  (50  to 
55  per  cent,  sugar,  10  to  15  per  cent,  dextrin,  5  per  cent,  albumin,  1 
tablespoonful  of  20  Gm.  is  equal  to  60  calories),  also  to  be  recommended 
as  a  mixture  with  milk  is  Loflund's  maltsoup  extract,  or  crj'stalhzed 
(Brunnengraber)  T\-ith  83  per  cent,  carbohydrates,  5  per  cent,  albumin; 
1  tablespoonful  equals  85  calories);  of  albuminoid  preparations:  puro 
(meat  juice  with  33  per  cent,  albumin,  19.16  per  cent,  extract);  of  milk 
albumin  preparations:  plasmon  (cheap),  nutrose,  sanatogen,  with  80  to 
90  per  cent,  albumin,  the  latter  also  with  5  per  cent,  glycero-phosphorate 
of  sodium,  all  soluble  or  miscible  in  cold  water;  also  the  inexpensive 
roborat  with  9-4  per  cent,  vegetable  albumin,  free  from  nuclein,  misci- 
ble in  water,  suitable  for  mixing  with  flour  for  making  bread  (up  to  40 
per  cent.)  somatose  Calbumose  with  SO  per  cent,  of  soluble  albumin)  is 
also  on  the  market  in  liquid  form,  it  is  expensive  and  in  large  quantities 
causes  diarrhcra.  Generally  albuminous  preparations  are  not  liked  for 
a  long-continued  period,  but  have  the  great  advantage  of  containing 
albumin  in  concentrated  form.  Meat  juice  (freslily  made  with  Klein's 
meat  juice  squeezer,  also  on  the  market  as  Valentine's  meat  juice,  etc.)  and 
meat  extract  may  be  given  as  appetizers,  but  have  little  nutritive  value. 

Medicinal  therapy  can  only  be  attended  with  success,  if  the  entire 
hygienic  measures  have  been  regulated  in  accordance  with  the  princi- 
ples laid  down  above.  At  all  events  the  administration  of  iron  for  sev- 
eral weeks  in  succession  is  always  advisable  as  soon  as  anaemia  has  once 
been  estabHshed. 


140  THE   DISEASES   OF   CHILDREN 

In  older,  flabby  children  arsenic  sometimes  produces  good  results; 
the  dose  is  to  be  gradually  increased  and  correspondingly  decreased. 

Not  infrequently  there  is  a  complication  of  scrofula  which  has  to  be 
treated  according  to  the  usual  methods.     Medication: 

Syr.  ferri  iodidi  (to  be  kept  from  the  light)  15-25  drops  three  times 
daily  (from  the  fifth  year). 

Headache,  want  of  appetite,  constipation,  generally  give  way  to 
the  hygienic  measures  mentioned  above.  For  headache  0.2.5-0.5  Gm. 
(Sj-?  grains)  antipyrin  may  be  given.  The  appetite  should  be  stimu- 
lated by  bitters,  such  as: 

Tr.  cliinchonje  comp.,  ^-1  teaspoonful  three  times  daily  (5-7-15 
years)  in  \  wineglass  of  sugar  water,  \  hour  before  eating;  tr.  rhei 
aromat.,  ^  teaspoonful,  same  way. 

The  following  is  also  good: 

Fellow's  syrup  three  times  daily  g-J  teaspoonful  (5-9-12  years)  in 
h  wTueglass  of  water,  then  orexin  in  chocolate  tablets  of  four  grains, 
several  days  once  or  twice  two  hours  before  meals. 

Constipation  may  be  benefited  by  just  taking  a  walk  especially  in 
children  with  weak  muscles;  useful,  however,  are  gymnastic  exercises, 
bending  of  the  body  (with  caution  at  time  of  menstruation),  breathing 
and  arm  exercises,  and  ma.ssage  by  a  skilled  operator.  A  glass  of  cold 
water  should  be  taken  in  the  morning  on  an  empty  stomach,  baked 
prunes,  also  coarse  food  (brown  whole  meal  bread),  buttermilk,  kefir 
two  days  old,  gingerbread,  malt  extract  (very  useful;  1-2  tablespoonful 
daily).  Care  should  be  taken  to  maintain  good  nutrition,  psychical  in- 
fluences should  not  be  overlooked,  for  even  young  children  may  be  pro- 
nounced hypochondriacs.  Regular  habits  should  be  inculcated,  daily  at 
the  same  time,  the  best  time  being  immediately  after  rising  or  after  the 
first  breakfast.  In  the  beginning,  artificial  help  should  be  resorted  to 
by  an  enema  of  a  pint  of  water,  two  ounces  oHve  oil,  or  an  injection  of 
1-2  teaspoonfuls  of  glycerin  with  double  or  treble  the  quantity  of  water. 
Medicinally  may  be  prescribed: 

Pulv.  glycyrrhizse  comp.  ^-1  teaspoonful;  pulv.  rhei.  comp.  by  the 
knife  point;  confection  of  tamarinds  (in  1  piece  0.5  senna),  ab.  i-i-1 
confection;  fluid  extract  rhamni  purshiana>,  2-3  times  daily  10-20 
drops  in  water  (bitter);  California  fig  syrup  (1  tablespoonful  contains 
3.11  senna)  one  teaspoonful  three  times  daily;  extr.  aloes,  extr.  rhei. 
aa  f.  pil.  1-2-3  pills,  (older  children). 

ANJEMIAS  AT  THE  END  OF  THE  NURSING  PERIOD,  INCLUDING 
ANiEMIA  PSEUD0LEUK.S;MIA  INFANTUM 

Nature  and  Name.— Among  the  wretched  children  who  sufl'er  from 
intestinal  disturbances,  rachitis,  etc.,  and  have  not  the  physical  strength 
of  healthy  children,  there  are  always  a  number  who,  after  the  period  of 


PLATE  7. 


PC  polychromatophile  ;  no.  normoblast  ;  me,  megalo- 
blast  ;  /,  lymphocyte;  np,  neutrophile;  ep,  eo--ino- 
phile  ;  n  my,  neutrophilic  myelocyte  ;  e  my,  eosino- 
philic myelocyte;   tho,  mononuclear-,   ma,  mast-celi. 


I.  Anaemia  pseudoleukeemia. — Fixation,  1^  formol-alcohol  ;  stain,  aqueous  eosin  sol.,  then  aqueous  methy- 
lene blue  sol.  Erythrocytes  of  different  size  and  form,  many  normoblasts,  megaloblasts,  and  mononuclear 
cell?.    Reds,  1,784.000;  whites.  30.200. 

II.  Anaemia  pseudoleukEcmia.  Stain,  May-Griinwald. — Light  poikilocytosis,  many  normoblasts  and  megalo- 
blasts. two  neutrophile  myeloc.\tes,  one  mast-cell. 

III.  AnEcmia  perniciosa  in  adults.  Stain  hzematoxylin  and  aqueous  eosin  sol. — Marked  poikilocytosis, 
megalocytes  (meM.  megaloblasts,  leucocytes  diminished. 

IV.  Chronic  myelogenous  leukaemia  (7-year-old  boy).  Stain.  May-Griinwald. — Great  increase  of  all 
nucleated  cells  with  differences  in  size.    One  giant  lymphocyte  (IM.     Reds  diminished. 

V.  Acute  lymphatic  leukaemia  1 13-year-old  boy).  Stain,  ha-matoxylin  and  aqueous  eosiu  sol. — Great 
increase  of  lymphocytes  of  different  sizes,  few  polynuclear  cells,  few  normoblasts. 


DISEASES   OF   THE    BLOOD  141 

nursing  has  come  to  an  end,  are  conspicuous  because  of  their  pale  com- 
plexion. The  majority  of  these  cases  are  cured  after  a  shorter  or  longer 
period,  but  in  some  cases  the  gravest  clinical  manifestations  develop. 
All  these  cases,  however,  have  so  many  points  in  common  by  their  time 
of  origin  at  the  end  of  the  nursing  period,  l)y  their  pathogenesis  and  not 
in  the  least  by  the  possibility  of  a  favorable  course  even  in  grave  blood 
changes,  that  it  seems  justified  to  consider  them  in  one  common  group. 

The  graver  cases  are  frequently  grouped  under  the  name  of  splenic 
anaemia  whicli  Itahan  authors  (Somma,  Jemma)  have  given  to  this 
symptom-complex  on  account  of  the  simultaneous  enlargement  of  the 
spleen;  or  it  is  called  pseudoleuka'mic  ana-mia.  This  name  was  pro- 
posed in  '1889  by  von  Jaksch  to  designate  a  pathological  picture  char- 
acterized by  oUgocytha^mia,  oligochromaMuia,  considerable  permanent 
leucocytosis,  enlarged  spleen,  slightly  enlarged  liver  and  sometimes  also 
enlarged  lymph-nodes.  The  term,  however,  is  not  very  happily  chosen, 
because  pseudoleuk;rmia  is  even  characterized  by  the  absence  of  increased 
leucocytosis. 

Itahan  authors  and  also  a  number  of  Germans  (Fischl,  Geissler, 
Japha)  include  the  grave  infantile  anu'inias  in  the  secondary  ana-mias 
with  a  toxic  basis,  while  others  regard  them  as  a  genuine  blood  disease 
(Aud^oud),  practically  as  a  middle  condition  between  anajmia  and  leu- 
kitMiiia.  Evidently,  different  authors  have  described  different  conditions 
under  the  same  name.  Cases  that  die  under  a  progressive  increase  of 
leucocytes  should  perhaps  be  classed  as  leukaemia  from  the  beginning. 
Some  authors  (Weil  and  Clerc,  Lehndorff)  prefer  to  separate  cases  with 
considerable  increase  of  myelocytes  (in  Lehndorff's  case  1.3-12.7  per 
cent.)  as  essentially  a  blood  disease  (perhaps  infantile  leukaemia).  True, 
in  Lehndorff's  case  the  organs  harbored  myeloid  foci,  but  there  are 
cases  with  numerous  myelocytes  (6.5  per  cent,  in  the  case  mentioned 
later  on)  whose  blood  findings  may  become  normal  again,  and  the  possi- 
bihty  of  a  favorable  termination  is  rather  a  point  which  might  induce  us 
to  include  these  cases  of  infantile  anajmia.  It  is  a  difficult  question 
whether  anaemia  pseudoleuka^mica  represents  the  pernicious  anaemia  of 
childhood  which  under  the  changed  conditions  of  cliildhood  shows  a 
somewhat  altered  blood  picture.  In  this  respect  the  observations  made 
by  Reckzeh  are  of  special  interest,  that  the  influence  of  blood  poisons 
in  young  animals  produces  a  blood  picture  which,  by  the  large  number 
of  normoblasts  and  leucocytes,  resembles  that  of  pseudoleukirmic  ame- 
mia,  whereas  in  full-grown  animals  the  picture  of  pernicious  antemia  is 
the  result.  It  is  questionable,  however,  whether  the  conditions  of  the 
animal  organism  can  be  applied  to  the  human  one. 

Etiology. — At  no  age  are  there  so  many  ana?mias  vath  relatively 
grave  blood  pictures  as  at  the  end  of  the  nursing  period,  say  from  the 
seventh  month  to  the  end  of  the  second  vear.    It  mav  therefore  be  as- 


142 


THE   DISEASES   OF   CHILDREN 


Fig.  21. 


sumed  that  at  this  particular  period  there  must  be  a  predisposition  for 
this  sort  of  affection.  Tliis  functional  deficiency  in  the  blood-forming 
apparatus  is  analogous  to  the  pecuhar  affections  of  the  osseous  and 
nervous  systems,  rachitis  and  tetany,  which  likewise  occur  at  this  pe- 
riod. Among  the  immediate  causes  there  are  in  the  first  place  artificial 
nutrition  and  its  sequela^,  nutritive  disturbances  and  intestinal  catarrhs 
(in  breast-fed  children  serious  cases  hardly  ever  occur),  unsanitary  and 
unhygienic  domestic  conditions.  Also  in  well-to-do  families  children  be- 
come affected  if,  for  fear  of  catching  cold,  they  are  kept  in  badly  venti- 
lated rooms.     Premature  children  are  particularly  exposed  as  well  as 

children  who  have  not 
completely  recovered 
from  some  haemorrhage 
(umbilical  haemorrhage, 
meliena).  The  statement 
that  tuberculosis  (Raud- 
nitz)  and  syphilis  exert 
a  predisposing  influence 
is  not  to  be  wondered  at, 
but  in  all  the  cases  ob- 
served by  the  author 
these  affections  have  not 
been  estabHshed  with 
certainty,  nor  are  grave 
ana-mias  a  particularly 
frequent  complication  of 
syphilis.  The  spleen  can- 
not be  the  primary  path- 
ological focus  and  can 
hardly  have  anything  to 
do  with  the  regeneration 
of  the  blood,  since  its  en- 
largement becomes  fre- 
quently reduced  before 
the  blood  picture  has  be- 
come normal,  while  the 
extent  of  the  enlargement  does  not  always  correspond  to  the  gravity  of 
the  anaemia.  Generally  deficient  new  formation  of  blood  seems  to  consti- 
tute the  nature  of  the  afTection.  Simultaneously  there  may  under  certain 
circumstances  be  an  exaggerated  destruction,  but  there  are  only  a  few 
cases  in  which  the  ansmic  (granular)  degeneration  of  the  red  blood 
corpuscles  is  found  as  positive  evidence  of  the  effects  of  a  blood  poison. 
Pathological  anatomical  findings  exist  only  in  limited  numbers 
even  of  the  severer  cases  of  anaemia.    In  these  cases  the  spleen  is  either 


An:rini:i  p-ruiiMlruk:l'niii:i  infantum.  Severr  1 
sideraljle  tlinkenmj;  of  tlie  epiph\'ses  of  the  lower  arms  anii  legs, 
considerable  bending  of  tlie  lower  arm  and  thigh,  considerable  thor- 
acic deformity,  frog  belly,  widening  of  the  inferior  thoracic  aperture: 
spleen  w-ith  distinct  incisure  reaches  to  the  symphysis;  liver  not 
particularly  enlarged. 


DISEASES    OF    THE    lU,OC)D 


143 


hard  or  soft  according  to  wlictlii'r  tlie  .stroma  or  parench3Miia  is  the  more 
affected  by  the  liypcr])hi.sia.  There  is  nearly  always  sHght  swelhng  of 
the  lymphatic  glands,  and  among  the  complications  there  is  chiefly 
bronchopneumonia.  Luzet  and  Lehndorff,  whose  case  may  perhaps 
have  to  be  classed  as  leuka-mia,  found  red  bone  marrow,  the  former  also 
cells  in  the  hepatic  parenchyma  which  he  regarded  as  precursors  of  red 
blood  corpuscles.    Lehndorff  found  myeloid  foci  in  the  liver  and  kidneys. 


Ancemia  pseudoleukreinica.  Oirl.  i-i  months  okl,  boy  10  montlis  old,  both  rachitic,  the  girl  a  syph- 
ilitic suspect.  Enlarged  spleen  with  but  slightly  enlarged  liver.  In  the  case  of  the  boy  the  incistire  of  the 
upper  margin  is  very  distinct. 


Blood-pigment  deposits  which  are  demonstrable  in  all  primary  blood 
destruction,  were  absent. 

The  symptoms  depend  upon  the  gravity  of  the  ana>mia.  In  milder 
cases  there  are  no  particular  complaints  except  a  gradually  increasing 
pallor  and  the  child,  which  does  not  thrive  and  is  usually  rachitic,  may 
be  quite  vivacious.  In  severer  cases  the  pallor  increases  and  assumes  a 
yellowish  tint;  the  child  becomes  weak,  ill  at  ease,  peevish,  hes  down 
listlessly,  and  answers  every  attempt  to  change  its  position  with  pitiful 
crying.  This  is  to  a  large  extent  occasioned  by  severe  rachitic  changes, 
and  besides  there  are  often  intestinal  disturbances,  and  a  large  percen- 
tage suffer  from  bronchopneumonic  affections  following  an  exceedingly 


144 


THE    DISEASES   OK   (HlLDliEN 


Fi.;,2S. 


chronic  course.     In  cases  of  this  kind  there  are  also  febrile  manifesta- 
tions.    Frequently  there  are  shght  ha-niorrhages  in  the  skin  and  mucous 

membranes,  less  frequently 
oil  tlie  surface,  especially 
cpistaxis.  Retinal  haemor- 
rhages are  hardly  ever  ob- 
served even  in  severe  cases. 
Among  the  objective 
signs  in  severe  cases  there 
is  always  an  enlarged 
spleen,  but  under  certain 
circumstances  only  so 
slightly  enlarged  that  it 
does  not  reach  beyond  the 
costal  arch  by  more  than 
1  to  ItV  finger's  breadth,  in 
other  cases  the  spleen  may 
reach  down  to  the  iliac 
crest  and  extend  by  sev- 
eral fingers'  breadth  be- 
yond the  median  line,  so 
that  its  outlines  are  dis- 
tinctly visible  through  the 
flabby  abdominal  wall 
when  the  child  hes  on  its 
back.  On  palpation  the 
organ  feels  strikingly  hard 
ami  the  incisure  of  the  up- 
per margin  may  often  be 
felt  distinctly.  As  a  mat- 
ter of  course  these  enlarge- 
ments distend  the  abdomen 
considerably,  widen  the 
inferior  thoracic  aperture 
and  disturb  the  movements 
of  the  thoracic  organs. 
The  Kver  is  seldom  much 
enlarged,  but  if  it  does  ex- 
tend by  two  fingers'  width 
beyond  the  costal  arch,  it 
generally  feels  soft .  Much 
swelling  of  the  lymphatic  glands  is  seldom  present,  but  nodes  the  size 
of  a  pea  or  bean  are  often  felt  in  the  neck,  axillfp  and  in  the  flexures 
at  the  elbow  and  groin. 


Acute  (  V  )  and  clironic  (  /  )  leukaemia.  Boy.  TA  year.s.  .sick  for 
months,  beginning  with  distention  of  abdomen  and  pain,  infantil- 
ism, hard  enlarged  spleen,  slightly  painful  on  pressure,  smooth, 
gradually  increasing,  only  temporarily  reduced  by  the  application 
of  X-rays.  Hiemoglobin  30-35  per  cent.  Erythrocytes  3,810,000, 
leucocytes 261,500,  L.  :  E.  1  :  14.5.  Blood  picture  (Plate  7.)  almost 
exclusively  granulating  cells,  among  which  are  found  mononuclear 
ones  in  large  quantity.  General  condition  not  serious,  boy  out 
of  bed  lively.    I.eukai'mic  retinitis. 

Girl,  3  years,  became  affected  a  few  weeks  ago,  beginning  with 
abdominal  complaints,  inability  to  walk,  enlarged  spleen  some- 
what softer  than  above,  deeply  notched,  of  changeable  size.  Grave 
general  condition,  haemoglobin  (Fleischl)  30  per  cent.,  E.  2.800.- 
000.  L.  1.250.000,  1..;  E.  1:  2.5.  Large  mononuclear  granulated 
cells,  mucous  membranes  slightly  discolored,  hcemorrhages  from 
no.se  and  skin,  also  from  vitreous  body,  grave  bilateral  leuksemic 
retinitis.     Both  children  still  unde.  treatment. 


DISEASES   OF   THE    BLOOD  145 

The  blood  findings  (see  Plate  7,  Figs.  1  and  2)  vary  according  to 
the  degree  of  the  ansemia  and  show  in  various  cases  every  gradation  to 
the  severest  lesions.  In  inild  cases  there  is  hardly  more  alteration  than 
a  diminution  of  the  ha>moglobin,  which  may  be  followed  later  by  a  more 
or  less  important  diminution  of  the  red  blood  corpuscles.  The  hiemo- 
globin  quotient  of  the  red  blood  corpuscles  is  diminished.  As  the  an- 
aemia progresses,  considerable  differences  in  the  size  of  the  red  blood 
corpuscles  are  noticeable,  forms  but  sHghtly  indented,  also  here  and 
there  nucleated  cells  of  normal  size  (normoblasts).  The  white  blood  cor- 
puscles in  mild  cases  without  compUcations  are  but  slightly  increased 
beyond  the  normal  number  for  the  infantile  period  (10,000  to  20,000); 
the  percentage  of  the  lymphocytes  corresponds  about  to  the  normal 
proportion  for  the  first  year  of  Ufe.  In  the  severest  cases  which  are  also 
classed  as  splenic  or  pseudoleuktemic  anteniia,  as  explained  before, 
the  hienloglobin  may  .sink  to  30  per  cent,  of  the  normal  and  lower  still, 
the  number  of  red  corpuscles  to  below  a  milhon.  The  amount  of 
haemoglobin  contained  in  the  separate  red  blood  corpuscles  may  vary 
considerably.  The  specific  gravity  may  be  materially  reduced  and  the 
tendency  to  mummular  formation  may  be  absent.  The  red  blood  cor- 
puscles exhibit  con.siderable  differences  in  size  from  dwarf  to  giant 
proportions,  sometimes  there  is  a  pronounced  poikilocytosis,  polychro- 
matophiha  and  in  isolated  cases  granular  degeneration.  Nuclear  forms 
are  sometimes  met  ^nth  in  such  large  quantities  as  hardly  in  any  other 
blood  affection,  up  to  20.000  in  a  c.mm.  (Lehndorff),  figures  of  many 
thousands  are  not  rare,  and  as  many  as  25  per  cent,  may  be  megalo- 
blasts.  There  is  a  considerable  increase  in  the  colorless  blood  cells  up 
to  50.000,  in  fact,  cases  have  been  reported  with  initial  leucocyte  counts 
of  114.510  (Jaksch)  and  122,222  (Baginsky)  which  have  been  cured. 
The  increase  concerns  rather  the  mononuclear  form,  so  that  the  per- 
centage of  polynuclear  neutrophile  cells  (30-40  per  cent.)  may  well 
appear  diminished  against  the  normal  infantile  figure;  the  proportion, 
however,  is  subject  to  changes.  Besides,  very  frequently  some  neutro- 
phile and  eosinophile  myelocytes  are  met  with^in  cases  which  are  Hke- 
wise  susceptible  to  improvement.  "Mastzellen"  exist  only  in  small 
quantities.  Often  there  are  strikingly  large  cells  with  round,  stainable 
nuclei  and  abundant,  coarse,  weakly  basophile  or  homogeneous  proto- 
plasm which  is  also  stainable  by  eosin. 

Nuclear  segmentations  occur  frequently,  especially  in  the  nuclear 
red  blood  cells,  but  also  in  the  white  cells,  and  they  can  be  well  seen  in 
the  Zeiss  chamber  in  a  blood  diluted  mth  a  1  per  cent,  acetic  acid.  In 
making  dry  preparations,  they  are  generally  crushed. 

The  follo^ving  table  contains  the  proportionate  figures  of  2  cases. 
In  the  first  case  (Fig.  21)  the  blood  picture  improved  considerably  by 
chnic  treatment;  death  ensued  later  during  home  treatment,  probablj'' 

11—10 


146 


THE   DISEASES   OF   CHILDREN 


from  bronchopneumonia;  the  second  case  was  completely  cured  in  the 
Children's  Asylum  of  the  City  of  Berlin  (Finkelstein);  the  child  made 
splendid    progress,  but  died  later  of  whooping-cough   and    pneumonia. 


1 

Poly  nuclear 
leucocytes. 

Mononuclear 
leucocytes 
(myelocytes) 

X 

ll 

5i; 

s 

5'c 

rt  g 

-I 

£ 

O 

3 

_a> 

— 

3 

£ 

§1 

-r  t-' 

a 

St 
■£?. 

'S 

« 

s 

c< 

•~ 

a 

o 

^ 

o 
a 

■&i 

^1 

i 

Eg 

c 

1 

it 

1 

o 

'A 

^ 

W 

tq 

H 

3 

>J 

« 

!« 

Cq 

i5 

>= 

:5 

Case   I  . . . 

30,200 

34.0 

4.9 

5.0 

1.5 

1.6 

49.0 

0.6 

0.6 

4. 

1:2.5 
leucocytes 

1:20 
leucocytes 

Large 
quantities. 

Case  II  . . . 

14,000 

40.0 

3.8 

4.9 

0.4 

3.2 

48.6 

1:5 

1:  22 

leucocytes 

leucocytes 

Course  and  Prognosis. — In  mild  cases  the  antemia  soon  improves 
under  proper  treatment;  in  severe  cases,  especially  if  external  injurious 
influences  cannot  be  removed,  it  persists  for  months  and  may  result  in 
death,  generally  in  consequence  of  coniphcations,  usually  of  broncho- 
pneumonia. Mild  cases  of  infantile  anaemia  should  also  be  taken 
seriously,  because  the  transition  to  severe  forms  is  never  impos- 
sible, and  at  all  events  under  insufficient  treatment  a  long-continued 
debility  may  remain  behind.  On  the  other  hand,  even  in  sevei'e 
cases  the  prognosis  need  never  be  pessimistic,  as  even  the  gravest 
blood  conditions  may  undergo  a  surprising  improvement  under  appro- 
priate treatment. 

The  diagnosis  itself  offers  no  difhculties.  The  differentiation  from 
pernicious  anaemia  is  possible  through  the  enlargement  of  the  spleen  and 
the  increase  of  leucocytes,  while  in  pernicious  anaemia  the  leucocytes  de- 
crease. Sometimes,  however,  the  differential  diagnosis  from  leukse- 
inia  is  by  no  means  simple.  For  splenic  anaemia  speaks  (1)  the  number 
of  leucocytes,  which  seldom  exceed  50,000;  (2)  the  practically  sustained 
percentage  during  the  first  year  of  the  various  kinds  of  leucocytes  in 
spite  of  the  presence  of  myelocytes,  with  a  possible  slight  preponderance 
of  lymphocytes;  (3)  the  considerable  changes  in  the  red  blood  corpus- 
cles, especially  the  enormous  quantity  of  nucleated  red  corpuscles.  The 
comparatively  small  size  of  the  liver  (von  Jaksch)  is  not  quite  a  reUable 
sign.  Doubts  may  exist  particularly  if  a  polynuclear  leucocytosis  com- 
phcates  the  anaemia,  because  in  that  case  the  blood  picture  sometimes 
resembles  myeloid  leuka-mia. 

The  best  prophylaxis  is  naturally  nourishment  from  the  mother's 
breast.  Artificial  nutrition  should  be  made  as  rational  as  possible.  It 
is  not  proven  that  a  particular  food,  such  as  buttermilk,  specially  fa- 
vors the  development  of  severe  anamiias,  but  any  such  food  had  better 
not  be  continued  too  long.  The  same  refers  to  the  feeding  \\ith  steri- 
lized preparations.  Of  importance  is  proper  regard  for  hght  and  air, 
even  in  winter. 


DISEASES   OF   THE   BLOOD  U7 

Treatment. — The  first  step  in  the  treatment  is  to  correct  errors  in 
diet  and  to  remove  intestinal  disturbances.  Of  importance  in  many 
cases  is  feeding  ^\•ith  mixed  diet,  such  as  a  few  teaspoonfuls  of  spinach, 
mashed  carrots,  mashed  potatoes,  asparagus  tips,  artichokes,  cauliflower, 
fruit  juice  (preferably  raw),  also  meat  juice,  broth,  and  the  exclusion 
of  oversterihzed  or  pasteurizetl  foods.  The  appetite  is  stinuilated  by 
the  administration  of  bitters  (tr.  cinchonte  conip.  or  tr.  rhei  vinos.,  8-10- 
12  drops  in  sugar  water  three  times  daily)  or  a  mixture  of  pepsin  and 
hydrochloric  acid  (1:2:100).  Great  care  should  be  taken  under  any 
circumstances  to  provide  good  light  and  air.  Children  of  poor  parents 
are  now  best  cared  for  in  children's  hospitals.  If  the  weather  conditions 
are  favorable,  infants  are  allowed  to  he  in  the  open  air  or  on  verandas. 
Children  living  in  cities  are  liighly  benefited  by  removal  to  a  dry,  sunny 
spot  in  the  country;  a  great  improvement  is  rapidly  produced  in  the 
case  of  poor  children  by  sending  them  to  the  garden  colonies  of  larger 
cities;  better  still  may  be  the  effect  of  a  mild  sea  chmate,  or  southern 
climates  in  winter.  Of  great  importance  is  the  care  of  the  skin:  it  should 
be  rubbed  with  flannels  and  spirits  of  calamus  several  times  e-\-ery  day, 
while  every  other  day  a  warm  chamomile  bath  or  one  jirepared  with  a 
decoction  of  calamus  should  be  given.  Wasliing  with  cold  water  should 
be  avoided.  At  the  same  time  direct  medicinal  treatment  may  also  be 
tried.  Rachitic  children  are  given  phosphorated  codhver  oil  to  improve 
the  anaemia  (phosphori  0.02,  ol.  jec.  aselli  ad.  100.0,  one  teaspoonful  in 
the  morning  after  the  first  meal,  in  all  about  three  bottles),  which  is  a 
suitable  combination  and  apparently  quite  efficacious.  Of  iron  prepa- 
rations the  following  are  suitable  for  infants:  hquor  ferri  alb.,  8-10-15 
drops  three  times  a  day;  ferri  lact.  0.03-0.05  Gm.  (J-f  gr.)  three  times  a 
day;  ferri  pyrophosph.  c.  ammon.  citrico,  0.05-0.1  Gm.  (|-li  gr.)  in 
mixture  three  times  a  day,  but  iron  may  also  be  dispensed  with.  Ai-senic 
is  sometimes  not  borne  well;  in  some  cases,  however,  the  use  of  greatly 
diluted  solution  (sol.  Fowleri  gtt.  2,  water  50.0  Gm.  (14  drams), 4-5  tea- 
spoonfuls  a  day,  for  a  child  of  2  years)  has  been  followed  by  remarkable 
improvement.  Heubner  recommends  a  teaspoonful  of  fresh  bone  mar- 
row three  times  a  day,  stirred  up  with  egg  or  spread  on  bread;  d'Orlandi 
recommends  25  Gm.  (7  drams)  daily  of  fresh  spleen  juice. 

CHLOROSIS 

Chlorosis  is  an  anaemia  wliich  attacks  almost  exclusivelj'  the  female 
sex  in  the  period  of  development  and  in  the  decade  following;  its  origin 
may,  however,  frequently  be  traced  to  early  childhood. 

Nature  and  Etiology. — Greatly  varying  theories  have  been  advanced 
on  the  nature  and  etiology  of  this  affection.  One  mistaken  idea  was 
that  it  owed  its  existence  to  a  disturbed  resorption  of  iron  (Zander),  to 
an  intoxication  from  the  intestine  (Duclos),  perhaps  in  consequence  of 
the  usuall}^  prevaihng  constipation;  Grawitz  attributed  the  cause  to  a 


148  THE   DISEASES   OF   CHILDREN 

primary  disturbance  in  the  formation  of  lynipli,  rendering  the  blood 
watery  and  swelhng  up  tlie  red  cells;  von  Noorden  considered  a  distur- 
bance of  the  inner  secretions  of  the  sexual  organs  the  cause  of  the  func- 
tional weakness  of  the  blood-making  organs;  Rosenbach  and  Meinert 
hold  that  the  corset  impedes  respiration,  displaces  the  abdominal  or- 
gans, and  thus  leads  to  nervous  and  functional  disturbances.  Virchow 
pointed  to  a  constitutional  cause.  In  individuals  who  have  shown  signs 
of  chlorosis  during  life,  he  found  hypoplasia  of  the  vascular  system 
(stenosis  of  the  aorta,  tlrinness  of  its  walls,  anomaUes  of  the  vessels). 
Even  if  all  cases  of  chlorosis  do  not  present  these  grave  irreparable 
changes,  the  origin  of  the  affection  is  nevertheless  probably  attributable 
to  a  constitutional  weakness  in  the  majority  of  cases.  Tins  \iew  is  sup- 
ported by  the  distinct  heredity  of  the  affection.  Tliis  weakness  may 
lead  to  disturbed  blood  formation  during  the  period  of  development, 
and  then  only  in  the  female,  such  disturbance  being  characterized  by 
the  fact  that  the  cells  do  not  sufficiently  assimilate  the  iron.  The  fact 
of  its  occurring  principally  in  the  female  has  its  analogy  in  the  occur- 
rence of  Basedow's  disease  at  the  same  period.  Tliis  fact  also  supports 
the  opinion  of  von  Noorden  who  looks  upon  the  affection  as  a  distur- 
bance of  the  internal  secretion  of  the  sexual  organs. 

Its  occurrence  may  be  favored  by  all  such  factors  as  may  be  regarded 
as  causes  of  secondary  ana-mia:  insufficient  food  supply,  ^^tiated  air  of 
school  rooms,  and — in  no  small  degree — the  objectionable  distribution 
of  school  time,  excessive  study,  insufficient  recreation  and  sleep;  in 
older  children  mental  excitement  through  homesickness,  worry,  anxiety, 
ambition  and  improper  literature.  Excessive  masturbation  is  probably 
much  more  to  l)e  regarded  as  a  sign  of  a  debilitated  constitution  than 
as  a  cause  of  injurious  effects. 

Symptoms. — The  blood  changes  in  chlorosis  are  characterized  by 
the  fact  that  the  hEemoglobin  is  disproportionately  decreased  to  a  much 
higher  extent  than  the  number  of  red  blood  corpuscles  (Duncan). 
According  to  Graeber  7  cases  out  of  28  had  more  than  5,000,000  red  cor- 
puscles, 13  cases  4  to  5  milhons.  A  reduction  of  the  red  corpuscles  by 
one  fifth  corresponds  to  a  sinldng  of  the  hajmoglobin  by  one  half.  For 
this  reason  the  red  corpuscles  look  pale  and  are  of  unequal  size.  In 
severe  cases  poikilocytes  and  normoblasts  may  finally  make  their  ap- 
pearance. The  white  blood  corpuscles  are,  according  to  reHable  authors, 
on  the  whole,  normal,  so  far  as  their  number  and  proportion  are  con- 
cerned. The  specific  gra^'ity  sinks  about  in  the  same  proportion  as  the 
percentage  of  ha:>moglobin.  It  should  be  remarked,  however,  that  the 
blood  changes  do  not  in  all  cases  keep  step  mth  the  chnical  symptoms; 
sometimes  it  is  a  matter  for  surprise  to  find  the  palest  complexion  asso- 
ciated T^ith  quite  a  sufficient  percentage  of  haemoglobin,  or  even  no 
blood  changes  at  all.  in  spite  of  pronounced  chnical  symptoms. 


DISEASES    OF    THE    BLOOD  149 

Nervous  troubles  are  the  dominating  factor  of  the  disease  picture. 
In  the  foreground  stands  lassitude.  In  mental  or  bodily  exertions 
there  may  be  flickering  before  the  eyes,  paroxysms  of  vertigo  and 
spells  of  fainting.  The  feebleness  often  stands  in  no  pro[)ortion  to  the 
blood  changes,  sometimes  it  is  only  pronounced  in  the  morning  hours, 
while  even  greater  exertions  can  be  borne  in  the  evening.  Anomalies  of 
disposition,  inchnation  to  ponder  over  things,  sensitiveness,  are  of 
frequent  occurrence.  There  may  be  pain  in  the  epigastrium  and  in  the 
sides  probably  owing  to  muscular  weakness;  palpitation  of  the  heart 
occurs  in  paroxysms  as  in  true  tachycardia.  Disturbances  of  the  vascu- 
lar innervation  are  also  frequent,  such  as  changes  of  the  complexion, 
cyanotic  extremities,  continuous  tormenting  feehng  of  chills,  tendency  to 
chilblains  in  winter.  Headache,  too,  which  occurs  partly  as  a  contin- 
uous dull  pressure  and  partly  as  paroxysmal  migraine,  may  to  a  certain 
extent  be  based  on  disturbances  of  vascular  innervation.  Dyspnoea 
is  frequently  caused  by  visibly  unskilful  and  far  too  flat  respiration. 
Complaints  about  digestion,  pains  in  the  stomach,  anorexia,  constipa- 
tion, always  play  an  important  role;  less  frequent  are  pecuUar  cravings 
(pica)  as  for  instance  for  eating  chalk,  coffee  beans  and  soured  food. 

The  numerous  complaints  correspond  comparatively  Uttle  with 
the  bodily  manifestations.  The  color  of  the  skin  and  mucous  membranes 
is  always  pale.  The  state  of  nutrition  may  be  good;  but  on  the  other 
hand  there  are  frequent  cases  of  badly  nourished,  chlorotic  young  women, 
especially  in  poor  famihes  if  its  beginning  dates  back  to  early  cliildhood. 
In  such  cases  a  general  enteroptosis  is  frequently  met  with,  also  in  girls 
who  do  not  wear  stays,  also  in  boys,  although  less  frequently.  The  en- 
largement of  tlie  spleen  described  in  chlorotic  cases  (45  per  cent,  accord- 
ing to  von  Noorden)  is  probably  to  be  regarded  as  partly  due  to  sinking. 

As  stated  above,  Virchow  looked  upon  the  cause  of  chlorosis  as 
a  hypoplasia  of  the  vascular  system.  Pronounced  cases  of  tliis  kind 
always  furnish  a  thstinct  pathological  picture  which  is  not  difficult  to 
diagnose  (Frantzel).  They  generally  occur  in  persons  of  tall  and  slen- 
der build  with  but  slightly  developed  muscles,  little  subcutaneous  fat, 
and  undeveloped  sexual  organs.  The  heart  may  be  enlarged,  the  car- 
diac impulse  increased,  the  arteries  strikingly  narrow  and  tense;  there 
is  an  absolute  incapacity  of  undergoing  even  sUght  exertions,  which 
defies  treatment.     The  affection  continues  for  years. 

The  heart  frecjuently  produces  a  spUt  first  sound,  or  even  a  systohc 
murmur.  The  increased  strength  toward  the  base  of  the  heart,  the  ab- 
sence of  increase  in  strength  of  the  second  pulmonic  sound,  the  venous 
murmur  over  the  jugular  veins  when  the  head  is  in  a  forward  position, 
are  indicative  of  its  functional  nature.  There  are  many  theories  about 
the  origin  of  the  abnormal  cardiac  sounds,  but  no  positive  explanation, 
whereas  the  venous   murmur  probably  arises  in  connection  with   the 


150  THE   DISEASES   OF   CHILDREN 

decreased  specific  gravity.  Cardiac  dulness  is  seldom  enlarged,  presuma- 
bly OTOiig  to  the  retraction  of  the  lungs;  this  is  also  said  to  be  shown  by 
X-ray  examination,  ffidema  is  not  frequent  in  cliildren.  As  mentioned 
already,  the  gastric  complaints  are  mostly  of  a  nervous  nature,  the  secre- 
tion of  acids  is  normal  or  more  than  normal,  assimilation  of  food  good 
(Lipman-Wulff).  Menstruation  is  generally  delayed  and  irregular,  fre- 
quently painful.  There  is  often  a  mucous  discharge  which  stains  the 
underclothing  barely  yellow.  Occurrence  of  acne  and  urticaria  is  con- 
sidered by  many  to  be  connected  with  chlorosis.  The  urine  is  normal  or 
increased  in  quantity;  when  improvement  takes  place,  there  is  said  to 
be  increased  diuresis,  according  to  von  Noorden.  The  latter  author, 
Eichhorst  and  Hayem  also  consider  fever  to  be  connected  mth  chlorosis. 

Among  the  compHcations  ulcer  of  the  stomach  may  occur  even  in 
quite  young  girls;  thrombo.sis,  however,  ought  to  be  rare  in  children. 
Advanced  anaemia  may  favor  the  development  of  scoliosis  in  growing 
indi^'iduals. 

Course  and  Prognosis. — Some  cases  of  chlorosis  take  an  acute  course 
in  otherwise  well  nourished  and  apparently  healthy  persons  and  are 
gradually  cured  under  proper  treatment  in  three  to  six  months.  This 
may  be  the  signal  for  a  permanent  cure,  but  there  may  still  lurk  a  ten- 
dency to  relapses  which  are  said  to  occur  principally  in  the  spring  or 
late  summer.  Chlorosis  which  has  commenced  in  early  childhood  may 
under  favorable  circumstances  and  proper  care  also  be  cured,  but  here 
a  prognosis  of  a  complete  return  to  health  is  uncertain.  It  is  just  in  these 
cases  that  in  later  years  hypoplasia  of  the  vascular  system  sometimes 
becomes  manifest,  or  else  there  is  a  tendency  to  relapses  and  develop- 
ment of  neurosis  in  more  advanced  age. 

The  diagnosis  requires  in  the  first  place  exclusion  of  all  organic 
lesions  especially  on  the  part  of  the  lungs,  intestinal  parasites  or  of  ulcer 
of  the  stomach,  which  lead  to  secondary  ana>mia  through  hspmorrhages, 
and  finally  of  renal  affections.  The  diagnosis  of  chlorosis  is  supported  by 
the  age  and  sex  of  the  patient,  the  history  of  the  case  and  by  the  decrease 
of  haemoglobin,  wliich  is  considerable  in  comparison  to  the  decrease  in 
red  blood  corpuscles.  In  pronounced  changes  of  the  blood  (considerable 
poikilocytosis,  normoblasts)  the  assumption  of  a  secondary  anemia 
always  suggests  itself.  The  only  question  is  whether  cases  where  the 
blood  changes  are  only  slightly  pronounced,  but  their  manifestations  are 
present,  should  be  classed  vdth.  chlorosis.  In  practice  tliis  has  formerly 
always  been  done,  and  perhaps  it  is  superfluous  even  now  to  carry 
through  a  distinct  separation.  The  pathological  picture  is  the  same  and 
perhaps  the  assumption  is  correct  that  blood  changes  are  more  in  the 
nature  of  a  symptom  which  occurs  in  female  persons  and  then  only  at  a 
certain  age.  For  purposes  of  therapy,  however,  the  examination  of  the 
blood  is  very  important. 


DISEASES    OF   THE    BLOOD  151 

Prophylaxis  commands  a  wide  and  grateful  field  in  the  prevention 
of  chlorosis,  because  under  proper  care  vicious  tendencies  undergo  an 
improvement  in  the  growing  organism.  The  principal  considerations 
are  care  for  sufficient  and  correct  nutrition,  the  correct  distribution  of 
work  and  recreation,  sufficient  stay  in  the  open  air,  and  abundant  sleep. 
Further  directions  on  tliis  subject  are  given  on  page  137.  It  should  be 
emphasized,  however,  again  and  again  that  the  growing  body  should 
not  be  compressed  into  a  narrow  corset. 

Therapy. — Mechcinal  therapy  is  of  special  importance  when  fairly 
marked  blood  changes  have  been  demonstrated.  The  iron  therapy, 
inaugurated  in  Germany,  by  von  Niemeyer,  has  retained  its  advocates 
in  spite  of  Bunge's  adverse  criticism. 

Now,  how  does  the  iron  take  effect?  The  fact  that  even  chlorotic 
patients  obtain  sufficient  iron  in  the  ordinary  mixed  diet  is  certain,  be- 
cause the  organism  contains  3  Gm.  of  iron,  the  feces  contain  0.007-0.008 
Gm.,  and  Hoffmann  estimated  the  daily  total  loss  of  iron  at  0.06  Gm.; 
and  tills  figure  may  still  be  too  liigh.  It  follows  that  the  cells  of  chlorotic 
patients  do  not  assimilate  sufficient  quantities  of  the  iron  contained  in 
the  articles  of  nutrition  in  the  shape  of  nucleo-albumin.  What  then  is 
the  effect  of  the  inorganic  iron?  Formerly  it  was  thought  that  it  was  not 
absorbed,  from  which  originates  the  theory  that  it  protects  the  organic 
iron  from  decomposition  by  combination  with  H,S,  and  that  it  exer- 
cises a  tonic  effect  upon  the  stomach.  But  the  ferric  nucleo-albumin  of 
the  food  by  no  means  undergoes  ready  decomposition,  and,  besides, 
iron  introduced  subcutaneously  was  supposed  to  have  a  beneficial 
effect.  Recently  the  fact  has  been  estabUshed  (Miiller)  that  also  the 
organic  iron  compounds  in  medicinal  doses  can  be  absorbed  and  intro- 
duced into  the  organism  by  way  of  the  general  circulation.  Indeed  they 
served  to  increase  the  amount  of  iron  in  experiments  on  animals  wliich 
had  been  deferrated  by  food  containing  but  Httle  iron;  it  was  even  an 
improvement  on  the  iron  contained  in  ordinary  food.  It  is  said  that  an 
increase  of  the  nucleated  red  blood  corpuscles  in  the  bone  marrow  was 
demonstrated,  which  was  regarded  as  showing  an  irritant  effect  upon 
the  bone  marrow.  On  the  other  hand,  to  supply  with  iron,  cells  wiiich 
did  not  possess  sufficient  iron  for  constructive  purposes  is  an  entirely 
different  matter  from  gi\ing  an  additional  iron  salt  to  chlorotic  persons 
whose  bone  marrow^  cannot  assimilate  a  naturally  sufficient  quantity. 
At  any  rate,  the  iron  therapy  has  obtained  a  secure  foundation  through 
the  recent  experiments,  and  it  is  probable  that  the  irritant  effect  of  the 
iron  upon  the  blood-forming  vessels,  which  had  been  assumed  in  theory 
to  exist  by  Harnack  and  von  Noorden,  exists  in  fact.  Possibly  also  the 
"fattening  with  iron"  acts  as  an  irritant. 

What  kind  of  iron  preparation  should  be  administered?  There  are 
two  kinds  to  be  considered:    (1)  those  which  are  changed  into  oxide 


15!2  THE   DISEASES   OF   CHILDREN 

salts  by  acids,  including  gastric  hydrochloric  acid,  and  into  this  category 
belong  metallic  iron,  oxide  salts,  protoxide  salts  and  ferric  albuminates 
or  peptonates;  (2)  compositions  more  lughly  constituted  and  more 
difficult  to  disintegrate. 

Formerly  it  was  said  that  the  organic  preparations  are  better  ab- 
sorbed and  the  building  up  of  haemoglobin  in  the  organism  is  facihtated. 
The  investigations  above  referred  to,  however,  are  in  favor  of  inorganic 
or  rather  such  iron  preparations  as  are  decomposable  in  the  intestine. 
Grawitz  reports  ha^vdng  observed  granular  degeneration  of  the  red  blood 
corpuscles  after  the  introduction  of  l^lood  preparations. 

Apprehensions  as  to  the  consequences  of  the  iron  therapy;  black- 
ening of  the  teeth,  heaviness  in  the  stomach  and  other  gastric  and  intes- 
tinal distui'bances,  do  not  seem  justified  when  sufficient  caution  is  used. 
.\11  iron  preparations  are  to  be  taken  on  a  full  stomach  -with  the  excep- 
tion of  chalybeate  waters  which  will  be  dealt  with  later;  chalybeates 
and  iron  tinctures  are  administered  through  a  glass  tube  and  the  mouth 
should  be  frequently  cleansed  and  rinsed  during  the  iron  treatment  on 
account  of  the  iron  deposit  within  the  mouth.  Should  the  feces  assume 
a  very  black  color,  Henoch  recommends  to  diminish  the  dose.  Fats  and 
acids,  however,  need  not  inspire  apprehension.  The  treatment  should 
last  from  4  to  6  or  8  weeks,  commencing  and  ending  gradually ;  if  necessary, 
the  treatment  is  to  be  repeated  after  four  weeks.  As  a  rule,  the  daily  dose 
for  the  adult  is  0.1  Gm.  (1^  grains)  metallic  iron  in  the  preparations  ;  chil- 
dren receive  less  in  proportion.  Character,  percentage  of  iron  and  dose 
are  shown  in  the  following  table  according  to  Quincke  and  von  Noorden: 

Inorganic  Iron  Preparations  and  Simple  Ferro-Albumin  Compositions. 
0.1  Gm.  metallic  iron  is  contained  in: —  dose  in  grams 

Ferrum  hydrogenio  reductum 0.1 

Ferrum  lactieum 0.5 

Ferrum  pyrophosph.  c.  amnion,  citr 0.55 

Ferrum  carb.  saccharat 10 

Tr.  ferri  acet.  aeth.  {Klaproth) 2.6 

Tr.  ferri  chlorat 2.8 

Ferrum  oxyd.  saccharat  soluh .3.6 

Iron  tropon 4.0 

Iron  somatose 5.0 

Tr.  ferri  clilorati  aether  {Bestiischej) 10.0 

Syr.  ferri  iodidi 1 1 .0 

Tr.  ferri  pomati 12.0-16.0 

Liq.  ferro-mangani  sacch.  u.  pept.  (Hclffenberg) 16.6 

Liq.  ferri  album 25.0 

Malt  extract  with  iron  {Loflund,  ferr.  pjTophosph.  c.  amnion,  citr.  2%).  .   27..5 

Tr.  ferri  conip 50.0 

Malt  extract  with  iron  {Schering,  ferr.  oxyd.  sacch.  solub.  3  %) 120.0 

Pil.  Blaudii  (0.02  Fe) 5  pil. 

PU.  aloet.  ferrat.  (0.03  Fe) 3-4  pil. 

The  tables  show  the  frequently  slight  percentage  of  the  higher- 
constituted  iron  compositions  which  often  probably  does  not  exceed  that 


DISEASES   OF   THE    BLOOD 


153 


of  the  blood  (and  therefore  also  of  the  blood  sausage).  The  conditions, 
however,  are  favorable  to  resorption,  and  all  these  preparations  have 
been  successfully  used.  The  table  also  shows  the  doses.  Aside  from 
the  recipes  mentioned  on  p.  139,  older  children  may  be  given  with  ad- 
vantage tr.  ferri  chlorati  aether.,  three  times  daily  10-15  drops,  and  1 
or  2  Blaud's  pills  three  times  daily. 


Iron  Compositions  not  Readilt  Decomposable. 


Compositiou. 


Carniferrin  - 


Triferrin  

Ferratin. 

Spinoferrin . . . 
Haemoglobin  . 
Fersaa 


Haemogallot  ■ 
Hamol 


Character. 


Composition    of    iron    with    carnophos- 
phoric  acid  (Nucleonj.  I 

ParaDucIein  and  iron ! 

Ferro-albumin  from  pig's  liver 


Dose  for  adults. 


3  X  0.3-0.5 


3  X  0.3-0.5 
3x0.5-1.5 


0.1  Gm.  inor- 
ganic iron  is 
contained  in. 


0.33  Gm. 


Extract  of  Ilieraoglobin  (Pfeuffer) 
Hsematogen  {  Hommel ) 


Blood  (human). 
Sanguiual 


Hsemalbumin  ■ 


3  times  1  teaspoon- 

ful. 

Blood-pigment  reduced  by  pyrogallol.  ...j  Twice  to  3  times 

I    5^-1  teaspoonful- 

Blood-pigment  reduced  by  zinc Twice  to  3  times 

3^-1  teaspoonful. 

Blood  preparation,  liquid 

Blood  preparal  ion,  liquid 


Blood  preparation,  liquid 3  times  M-1  table- 
spoonful. 
Blood  preparation,  liquid 3  times  J4-1  table- 


3  times  %-l  table- 
spoonful. 


spoonful. 


71, 
142 


166. 
250. 


The  subcutaneous  injection  (in  the  adult  a  5  per  cent,  solution  of 
ferrum  citricum  oxyd.,  0.05-0.1  c.c.  (rT\,f-l2)  into  the  nates;  as  recom- 
mended by  Glaevecke,  and  Quincke)  will  hardly  find  application  in  the 
child;  it  causes  smarting  at  the  point  of  injection  for  24  hours;  rectal  in- 
troduction (ferr.citr.oxyd.  0.1-0.6  c.c.  (in,  lJ-9)  in50c.c.(-|-  IJoz.)  starch 
solution,  three  times  daily,  after  Jolasse)  is  Hke\Aise  hardly  indicated. 

Some  excellent  authors  attribute  a  special  influence  to  chalybeate 
waters  (Henoch,  von  Noorden,  Senator);  their  importance  lies  proba- 
bly to  a  certain  extent  in  the  very  liigh  attenuation  of  the  iron  (0.01 
to  not  more  than  0.1:1000),  also  in  the  fact  of  their  holding  CO,  in 
solution  and  in  the  possibility  of  administering  the  same  on  an  empty 
stomach.  If  it  is  used  at  the  springs,  there  are  of  course  many  other 
influences  to  be  considered. 

The  saUne  carbonated  waters  are  said  to  have  the  best  effect  proba- 
bly on  account  of  their  purgative  effect  in  constipation.  Of  the  non- 
arsenious  ones  1  pint  is  given  (warmed,  if  desired)  on  an  empty  stomach 
in  the  morning,  one  pint  and  a  half  ^^^th  the  dinner,  and  the  same  quan- 
tity six  hours  after  the  principal  meal.  For  home  use  pyrophosphorated 
iron  water  is  more  suitable  becau.se  even  with  the  greatest  care  in  filling 
the  bottles  the  iron  is  lost  (Binz). 

Should  iron  not  have  the  desired  effect,  ar.senic  may  be  given  to 
advantage,  especially  in  weak-muscled  children  with  enteroptosis.  The 
doses  are  given  on  p.  140  and  the  admissible  ma.ximum  dose  is  1  mgm. 
(?T  gr.)  daily.    Arsenious  chalybeates  are,  of  course,  suitable  in  these  cases. 


154  THE   DISEASES   OF   CHILDREN 

Many  physicians  at  the  present  time  assist  or  replace  iron  medica- 
tion by  diaphoretic  measures,  prescribing  a  hot  bath  two  or  three  times 
a  week,  followed  by  an  hour's  sweating,  or  else  the  use  of  the  Phenix 
hot  air  apparatus  (Grawitz,  Rosin,  Senator,  Mamlock).  Raebiger  has 
made  two  series  of  experiments,  one  exclusively  with  iron  medication  and 
one  exclusively  with  diaphoretics.  The  success  of  the  second  series  was 
as  large  as  that  of  the  first.  It  is  not  certain,  however,  whether  this  is 
due  to  the  water  economy  of  the  system  or  to  the  general  effect  upon  the 
metaboUsm.  Cold  hydriatic  measures  should  be  avoided,  the  remarks 
made  in  regard  to  school  ana-mia  (p.  135)  applying  to  these  cases  Hke- 
wise.  On  the  other  hand  it  is  a  very  good  plan  to  accustom  the  body  to 
colder  temperatures  by  the  use  of  carbonic  acid  baths,  of  wliich  3  should 
be  taken  weekly  (Senator  and  Frankenhiiuser) ;  also  the  effect  of  so- 
called  mineral  baths  is  probably  based  upon  their  containing  CO^,  the 
iron  they  hold  not  being  resorbed  by  the  skin. 

In  regard  to  other  dietetic  methods,  nutrition,  good  nursing,  dura- 
tion of  sleep,  gymnastic  exercises  (respiration),  sport  and  games,  the 
same  remarks  apply  which  were  made  in  regard  to  school  anamiia,  also 
the  remarks  in  regard  to  the  treatment  of  complications.  Chilblains  are 
favorably  influenced  by  long  bathing  of  the  extremities  in  hot  water 
with  a  httle  alum,  painting  with  ichthyol  coUodion  (10  per  cent.),  inunc- 
tion with  camphorse  tritse  5.0  c.c.  (1  dr.)  vasehni  ad  50.0  c.c.  (10  dr.). 
The  discharge  oozing  from  the  vagina  is  hardly  debihtating  as  is  gen- 
erally supposed;  in  these  cases  as  well  as  in  menstrual  troubles,  local 
treatment  should  be  warned  against   (aspirin,  antipyrin). 

PERNICIOUS   AN.ffiMIA 

Nature,  Etiology. — The  name  of  Pernicious  Amrmia  designates  an 
affection  in  wliich  there  is  progressive  diminution  and  degeneration  of 
the  red  blood  corpuscles,  usually  associated  with  fatty  degeneration  of 
internal  organs. 

Lebert  (1852)  and  Addison  (1855)  had  already  described  the  patho- 
logical picture  of  severe  anamiic  conditions  as  a  special  kind  of  anaemia, 
and  Biermer  (1864)  established  its  chnical  hues  of  demarcation.  The 
pathological  anatomy  of  the  bone  marrow  was  described  in  detail  by 
Cohnheim  in  1878,  while  EhrUch  at  a  later  period  gave  an  exact  descrip- 
tion of  the  blood  changes. 

Formerly  a  distinction  was  made  between  pernicious  anaemia  with 
a  known  cause  and  a  cryptogenic  pernicious  anaemia,  but  more  recently 
some  authors  (Grawitz,  Lazarus)  are  inchned  to  look  upon  the  entire 
group  as  secondary  disturbances,  although  there  may  be  a  difference 
in  the  congenital  capacity  of  the  blood-forming  organs.  The  following 
causes  have  been  observed:  Chronic  poisoning  (carbon  monoxide, 
Laache),   tumors,   especially   of   the   bone   marrow,  infectious   diseases 


DISEASES    OF   THE    BLOOD  155 

(sepsis,  syphilis,  malaria),  bodily  and  mental  injuries,  disturbances  of  the 
digestive  tract  (autointoxications)  and  perhaps  repeated  small  hirmor- 
rhages.  During  pregnancy  the  affection  is  comparatively  frequent. 
The  best  investigated  kind  is  parasitic  anajmia  caused  by  bothriocephalus 
latus  (Schaumann  Jind  Tallquist),  anchylostoma  duodenale  (Zinn  and 
Jacobi),  also  by  ascarides  lumbricoides  (Demme).  There  is  always  a 
destruction  of  blood  in  this  affection,  as  is  evident  from  the  large  amount 
of  iron  contained  in  the  internal  organs,  especially  the  hver,  urobiUnuria, 
manifestations  of  (nuclear)  degeneration  of  the  red  blood  corpuscles 
(Grawitz),  but  the  bone  marrow  suffers  secondarily  an  obstinate  and 
perhaps  permanent  change  of  function.  EhrHch  regards  the  change  of 
the  blood-forming  function  as  anatomically  characterized  by  the  devel- 
opment of  megaloblasts  (especially  large  nucleated  red  blood  corpus- 
cles) in  both  bone  marrow  and  blood;  others  do  not  consider  this  as 
specific,  but  only  as  an  expression  of  the  gravity  of  the  ansemia. 

Cases  occurring  in  children  have  been  described,  but  the  affection 
is  very  rare  in  children.  Many  factors  which  are  regarded  as  causative 
in  the  adult,  enter  rarely  or  not  at  all  into  consideration  with  children 
(pregnancy,  psychic  depression,  tumors);  it  is  also  possible  that  the 
bone  marrow  of  the  child  reacts  differently. 

Lazarus  found  among  240  rejjorted  cases  1  in  the  first  decade  (8 
young  girls  by  H.  Miiller)  and  22  in  the  second  decade.  Then  follow  11 
cases  compiled  by  Monti  and  Berggri'in,  6  by  Escherich,  2  by  Grawitz 
(children  of  12  and  8  years  respectively),  3  by  Koren,  1  by  Theodor,  1 
by  Mott  (a  9-months-old  girl),  3  caused  by  anchylostoma  by  Baravallo, 
Villa,  Cima.  These  few  cases  have  not  even  been  described  with 
accuracy,  some  can  hardly  be  accepted  as  true  pernicious  antemia 
(Baginsky,  1  case  by  Retslag),  others  are  doubtful  (Senator).  However 
that  may  be,  a  few  certain  cases  have  been  observed  even  in  the  first 
year  of  hfe. 

Symptoms. — The  subjective  complaints  of  children  are:  lassitude, 
weakness,  headache,  fainting  spells,  nausea,  gastric  pains,  anorexia. 
Pains  in  the  bones  such  as  occur  with  adults  in  the  tibia  and  sternum 
have  not  been  mentioned  in  the  case  of  children.  Objective  symptoms 
are  the  foUo^Ning:  sallow  complexion,  fat  cushion  sometimes  well  pre- 
served, frequently  oedema  of  the  legs,  haemorrhages  in  the  skin  of  various 
extent,  haemorrhages  in  the  mouth,  also  retinal  haemorrhages  at  an 
early  stage;  intestinal  haemorrhages  ha»ve  been  observed  comparatively 
frequently  in  children.  The  body  temperature  may  be  normal,  in  some 
cases  however  it  is  considerably  raised,  pulse  frequent,  respiration  dys- 
pnoeic.  The  heart  shows,  aside  from  visible  palpitation  and  pulsation  of 
the  carotids,  sometimes  enlargement  to  the  left  and  right,  and  especially 
murmurs  which  may  be  diastolic  and  cause  the  distinct  impression  of 
cardiac  insufficiency.     Venous   murmurs   may   be   present.      Diarrhoea 


156  THE   DISEASES   OF   CHILDREN 

occurs  frequently.  Severe  disturbances  of  consciousness  also  occur  in 
children,  but  paralysis  (from  poisoning  or  anatomic  changes)  has  not 
been  observed.  The  spleen,  usually  enlarged  in  adults,  is  often  swollen 
in  children,  but  this  may  be  the  consequence  of  a  complication.  The 
urine  in  the  adult  is  usually  dark  antl  often  contains  much  indican 
(increased  decomposition  of  albumin,  Grawitz),  also  urobiUn  as  a  con- 
sequence of  blood  destruction.  The  oxygen  consumption  and  the 
nitrogen  metabolism,  and  also  absorption,  are  frequently  entirely  undis- 
turbed, provided  there  is  light  diet;  Rosenquist  in  a  bothriocephalus 
anaemia  observed  in  the  disease  periods  of  increased  decomposition  of 
albumin  alternating  with  normal  conditions.  The  blood  (Plate  7)  is 
watery,  of  low  specific  gravity  and  considerably  diminished  coagula- 
bihty.  Hffimoglobin  and  red  blood  corpuscles  are  reduced  to  a  minimum, 
and  yet  the  coloring  power  of  the  individual  corpuscle  may  at  the  same 
time  be  normal  or  even  increased.  This  manifestation,  however,  can 
hardly  be  looked  upon  as  specific.  The  white  blood  cells  are  relatively 
and  absolutely  reduced  in  numbers  (although  in  one  case  of  Grawitz 
50,000  were  counted  in  a  child),  the  proportion  to  the  red  lilood  cor- 
puscles in  the  adult  is  reduced  to  1  :  1200  to  1600,  and  according  to  the 
majority  of  observations  the  lymphocytes  have  the  highest  percentage 
(up  to  60  per  cent.).  The  red  blood  cells  do  not  exhibit  any  mummular 
formation,  there  is  a  strongly  developed  poikilocytosis,  exceedingly 
small  (dwarf  corpuscles)  and  sometimes  very  large  forms  (megalocytes), 
further  polychromatic  and  nuclear  degeneration,  associated  with  mani- 
festations of  regeneration  (nuclear  normoblasts  and  megaloblasts). 

Anatomy. — Autopsy  shows  :  enormous  pallor  of  all  internal  or- 
gans, ha-morrhages,  especially  in  the  serous  membranes,  fatty  degenera- 
tion, especially  pronounced  in  the  cardiac  muscle,  siderosis  of  the  liver 
(haemosiderosis),  in  some  cases  atrophy  (anadenia)  of  the  gastric  and 
intestinal  mucous  membranes  which  however  may  not  be  regarded  as  a 
specific  manifestation  of  the  affection,  transformation  of  the  yellow  mar- 
row of  the  long  tubular  bones  into  red  marrow,  which  may  also  occur  in 
other  anemias  (E.  Neumann),  whereas,  on  the  other  hand,  yellow  mar- 
row may  be  found  in  pernicious  anaemia  (aplastic  marrow,  Ehrlich),  in 
which  case  there  should  be  no  nuclear  cells  in  the  blood.  The  red  marrow 
of  pernicious  anemia  is,  according  to  Ehrlich,  megaloblastic. 

The  course  of  the  disease  may  be  subacute  or  decidedly  chronic, 
frequently  there  are  also  relapses  follo\\ing  improvements.  Death 
ensues  in  coma  o^dng  to  progressive  weakness. 

The  prognosis  depends  partly  upon  the  cause.  Can  the  latter  be 
removed,  as  in  the  case  of  intestinal  parasites,  the  affection  is  amenable 
to  treatment.  But  also  in  the  cases  of  obscure  origin  the  prognosis  is 
not  absolutely  unfavorable  under  appropriate  therapy,  a  point  again 
and  again  emphasized  by  Grawitz. 


DISEASES    OF    THE    BLOOD  157 

The  diagnosis  of  pernicious  anajmia  is  made  by  the  hsemorrhages 
in  the  retina,  possibly  by  a  demonstration  of  urobilinuria,  and  finally 
by  the  blood  examination.  In  children  over  four  years  old  and  in  adults 
the  demonstration  of  megaloblasts  is  important,  but  not  in  younger 
children.  Against  a  diagnosis  of  pseudoleuksmic  anaemia  speak  a  dim- 
inution of  the  white  blood  cells,  excessive  diminution  of  the  red,  and 
perhaps  a  considerable  poikilocytosis;  on  the  other  hand  nuclear  red 
cells  exist  in  large  numbers  in  pseudoleuka^mic  antemia. 

The  therapy  should  pay  particular  attention  to  etiological  condi- 
tions: the  first  thought  should  be  of  intestinal  parasites;  Lazarus'  ad- 
vice to  administer  an  antiparasitic  medicine  (extract  of  male  fern)  even 
where  no  eggs  can  be  detected,  seems  well  worthy  of  consideration;  the 
second  thought  is  of  syphihs  (osteosclerosis),  although  the  therapy 
(iodine  and  mercury)  does  not  seem  to  hold  out  many  chances  in  these 
cases;  in  the  third  place  stand  occult  hsemorrhages  especially  in  the 
gastro-intestinal  canal — there  are  very  exact  methods  of  examination 
now  to  detect  blood  in  the  gastric  contents  and  in  the  feces;  in  the 
fourth  place  should  be  mentioned  the  possibility  of  chronic  effects  of 
carbon  monoxide  and  lead;  in  the  fifth  place,  the  gastro-intestinal 
canal  should  be  treated  by  irrigation  of  the  stomach  and  intestine, 
administration  of  hydrochloric  acid,  salol,  calomel,  or  better  by  bitters, 
etc.  The  diet  should  be  easily  digestible,  very  nutritious  (albuminoid 
with  vegetables  preponderating  in  the  beginning).  The  appetite  should 
be  stimulated  and  care  taken  to  provide  rest,  hght  and  air.  There 
is  only  one  medicine  which  is  sometimes  attended  with  excellent  results, 
and  that  is  arsenic  in  drops  or  pills.  Children  of  8-15  years  receive  up 
to  i— f  of  the  maximum  adult  dose;  or  it  maybe  injected  subcutaneously. 
According  to  Ziemssen's  method  1  Gm.  (15  grains)  arsenic  is  dissolved  in 
5  c.c.  (1  dr.,  ni  15)  boihng  normal  soda  solution,  then  distilled  water  is 
added  to  make  100  c.c.  (3  oz.,  2  dr.),  and  filtered;  in  adults  a  sterile 
injection  is  made  of  0.001  c.c.  (ni,  J^)  up  to  0.01-0.02  c  c.  (n^  |-J)  daily; 
or  sterilized  subcutaneous  injections  may  be  made  of  sodium  cacodylicum 
Merck  in  doses  of  0.05-0.1  c.c.  (n^  1-1  J)  in  adults.  It  is  always  neces- 
sary to  commence  ^\^th  very  small  doses,  increasing  or  diminisliing  the 
same  slowly,  this  being  the  method  to  avoid  intoxicating  phenomena. 
Among  the  latter,  pigmentation  of  the  skin  is  without  importance,  while 
susceptibiUty  of  the  buccal  mucous  membrane,  gastric  pains,  diarrhoea, 
oedema  of  the  eyelids,  herpes  zoster,  would  demand  at  least  a  temporary 
inhibition  of  the  medication,  although  it  i.s  perfectly  possible  that  these 
symptoms  may  partly  be  occasioned  by  the  antcmia. 

Finally  the  injection  of  small  quantities  of  defibrinated  blood  in 
very  grave  cases  should  be  considered.  Surprising,  although  unfortu- 
nately only  transitory  results  (Quincke,  Ewald)  have  been  attained  by 
the  introduction  of  small  quantities  (40  c.c),  the  cause  of  which  is  prob- 


158  THE   DISEASES   OF   CHILDREN 

ably  to  be  found  in  the  extreme  reaction  of  the  organism  to  its  intro- 
duction (Bier),  as  shown  by  severe  manifestations,  high  fever,  etc. 

LEUKJEMIA 

Nature  and  Etiology. — Lcuku'mia  is  characterized  by  an  excessive 
increase  of  the  wliite  blood-cells,  swelling  of  the  lymphatic  and  blood- 
forming  vessels,  and  finally  by  the  occurrence  of  lymphatic  neoplasms 
in  the  organs. 

Virchow  was  the  first  to  correctly  recognize  the  nature  of  the  affec- 
tion, demonstrating  that  there  is  an -increase  of  leucocj'tes,  whereas 
Bennet  had  thought  of  a  kind  of  pus  fermentation  of  the  blood.  Neu- 
mann ])articularly  achieved  considerable  advance  by  his  work  on  the 
l)lood-forming  function  of  the  bone  marrow,  wliile  Ehrhch  succeeded  in 
perfectly  differentiating  the  cells  by  proper  staining  methods. 

Observations  of  leukaemia  without  anatomical  findings  are  not 
quite  safe  (Hirschlaff),  and  Loe^\•it's  findings  of  amoebse  in  leuka}mic 
blood  have  not  been  confirmed  by  others.  On  the  contrary,  the  majority 
of  authors  attribute  leukaMnia  to  a  pathological  condition  of  the  blood- 
forming  vessels.  Formerly  a  distinction  was  made  between  leuka-mia 
lymphatica  and  leukemia  Uenahs,  and  when  Neumann  in  1866  dis- 
covered the  fact  that  to  the  bone  marrow  belonged  the  function  of 
forming  the  blood  corpuscles,  there  was  added  a  new  kind:  leuktemia 
medullaris.  Later,  in  1878,  Neumann  proved  that  in  every  case  of  leu- 
kaemia the  bone  marrow  was  involved,  and  therefore  assumed  (as  did 
also  Walz  and  Pappenheim)  a  myelogenous  origin  for  all  cases  of 
leukaemia.  On  the  other  hand,  Ehrlich  and  his  disciples  held  that  the 
lymphoid  tissue  is  also  to  be  considered  as  a  source  of  origin,  and  there- 
fore distinguishes  between  lymphatic  leukaemia,  caused  by  prolifera- 
tion of  lymphoid  tissue  (which,  by  the  way,  may  according  to  Pincus 
have  its  principal  seat  not  only  in  the  lymphatic  glands,  but  also  in  the 
lymphoid  part  of  the  medulla,  the  spleen  or  intestine)  and  myelogenous 
leuksemia,  caused  by  proliferation  of  tlie  typical  medullary  tissue. 
Clinically  both  forms  are  distinguished  by  the  blood  findings,  which  dis- 
close in  the  first  form  principally  lymphatic  cells,  and  in  the  second  gran- 
ulated (medullary)  cells.  According  to  Ehrlich  the  difference  is  material, 
because  the  immobile  cells  of  the  first  group  can  only  be  introduced  into 
the  blood  by  being  passively  swept  away  from  the  blood-forming  or- 
gans, whereas  in  the  second  form  there  is  active  leucocytosis.  Accord- 
ing to  some  authors,  however  (especially  Grawitz),  the  so-called  lym- 
phatic cells  of  lymphatic  leukteniia  are  partly  nothing  but  early  stages 
of  development  of  medullary  cells  (i.e.,  really  juvenile  forms,  as  A. 
Frankel  expressed  himself  in  opposition  to  Ehrhch).  The  lymphatic 
swelUngs  of  the  organs  are  perhaps  to  be  partly  considered  as  new 
formations    (metastases),   in    many   cases    however    as    hyperplasias    of 


DISEASES   OF   THE    BLOOD.  159 

pre-existing  lymphatic  foci.  The  ciuestion,  however,  whether  the  myeloid 
foci  also  originate  through  transformation  of  pre-existing  lymphatic 
ones,  is  doubtful. 

Following  Ehrlich's  initative  the  forms  of  leukaemia  are  now  gen- 
erally distinguished  as  lymphatic  and  myeloginous  (myeloid,  Pincus, 
mixed-celled,  Pappenheim,  Grawitz),  according  to  the  cells  present  in 
the  blood.  The  former  is  usualh'  acute,  although  there  are  also  chronic 
cases;  the  latter  mostly  chronic  although  there  are  rare  acute  cases  too 
(Hirschfeld,  Alexander,  Grawitz). 

Etiology. — A  parasitic  etiology  was  supposed  to  exist,  but  not  veri- 
fied. The  fincUng  of  a  plasmodium  by  Loewit  has  not  been  confirmed 
by  others.  An  infectious  cause  is  probable  in  some  cases  according  to 
A.  Frankel  on  account  of  the  enlargement  of  the  lymphatic  glands  of 
the  neck  in  the  first  instance,  and  then  by  the  subsequent  course.  But 
it  is  by  no  means  certain  that  there  is  a  uniform  cause  for  leuksemia. 

Bone  tumors  may  hkewise  lead  to  a  leukaemic  blood  picture,  espe- 
cially chloroma,  which  has  derived  its  name  from  the  green  color  of  the 
tumors.  The  affection  is  most  often  found  in  children  and  young  people. 
The  seat  of  the  new  formation  is  preferably  the  periosteum  of  the  cra- 
nial and  trunk  bones,  but  all  the  lymphatic  organs  may  become  involved. 
One  of  the  earliest  noticed  symptoms  is  exophthalmos.  Hsemorrhages 
and  manifestations  of  rapid  leuksemia  supervene  and  the  disease  termi- 
nates fatally  (Rosenblath,  Risel). 

As  predisposing  causes  are  considered  sj'phiHs,  long-continued 
malaria,  diphtheria,  membranous  angina,  influenza,  trauma;  co-existing 
tuberculosis  has  also  frecjuently  been  observed.  Rare  cases  of  infec- 
tion and  heredity  have  been  reported. 

Leuka?mia  generally  attacks  persons  in  the  best  years  of  fife,  but 
juvenile  cases  have  likewise  been  observed.  The  male  sex  is  chiefly 
attacked. 

After  Montr  and  Berggriin,  Grawitz,  Pinkus,  and  Lustgarten  had 
reported  cases  in  children,  there  have  appeared  more  recent  accounts 
by  Bauer,  Berghiinz,  Guinon  and  Jolly,  Jeanselme  and  E.  Weil,  Kelly,  E. 
Miiller,  Pollmann,  Rocaz,  Savory,  Strauss,  and  "\'ermehren;  a  case  of 
A.  Frankel  was  that  of  a  boy.  A  few  pecuhar  cases,  the  symptoms  of 
which  resembled  those  of  pernicious  antemia,  were  described  by  Arneth- 
Leube,  Geissler,  Japha  and  Scharlau.  Quite  young  children  were  af- 
fected in  the  cases  of  Pollmann,  Strauss,  Vermehren,  in  one  by  the 
author,  and  also  in  the  cases  of  Bloch  and  Hirschfeld,  Lehndorff,  which 
are  somewhat  dubious  as  to  classification.  In  the  first  four  cases  the 
number  of  leucocytes  is  so  high  (Japha  361.000)  that  the  existence  of 
a  true  leukaemia  can  hardly  be  doubted,  diflicult  though  the  chagnosis 
in  infants  may  be.  Chronic  myeloid  were  the  cases  of  Berghiinz  (8- 
year-old  girl)  and  Fleisch,  a  case  from  the  Gratz  Klinik  (Pfaundler,  7 


160  THE   DISEASES   OF   CHILDREN 

years)  and  the  author's  case,  the  course  of  which  was  somewhat  sub- 
acute. As  a  rule,  however,  the  acute  lymphatic  cases  are  the  most 
frequent  in  childhood. 

The  anatomical  examination  discloses  a  more  or  less  considerable 
swelling  of  the  lymphatic  glands,  the  spleen  and  the  lymphoid  follicles 
of  the  digestive  tract.  Besides,  yellowish  wliite  foci  may  exist  in  all 
the  other  organs;  the  liver  especially  is  usually  considerably  enlarged. 
The  adipose  marrow  is  of  red  color  (lymphadenoid),  but  not  corre- 
sponding to  the  normal  red  marrow,  or  of  a  deliquescent  nature  vaih. 
cells  of  the  medullary  type.  There  are  also  ha'morrhages.  The  cellular 
foci  in  the  organs  con.sist  of  lymphoid  cells,  there  is  no  necrosis.  In  the 
blood-forming  organs  the  microscope  reveals  only  a  hyperplasia  of  the 
normally  existing  elements,  but  in  lymphatic  leuka-mia  the  bone  mar- 
row consists  almost  exclusively  of  lymphatic  cells,  while  in  myelogenous 
leukaemia  a  myeloid  degeneration  of  the  spleen  and  lymphatic  glands 
has  been  described. 

Symptoms. — The  affection  commences  suddenly  or  gradually  with 
weakness,  lassitude,  anorexia,  pains  in  the  limbs  or  bones,  especially  in 
the  left  side  (spleen).  Mild  or  severe  fever  develops,  the  sallowness  of 
the  complexion  increases,  there  is  enlargement  of  the  glands,  spleen  and 
hver,  also  hfemorrhagic  diathesis.  As  the  dropsical  manifestations 
increase,  death  ensues,  frequently  caused  by  secondary  septic  involve- 
ments especially  on  the  part  of  the  tonsils,  ulcerative  stomatitis,  hypo- 
stasis and  pneumonia. 

The  blood  is  strikingly  pale,  clay-colored  or  milky.  The  determina- 
tion of  haemoglobin  is  rendered  difficult  by  the  increase  in  wliite  cells. 
In  all  cases  there  is  an  increase  of  the  colorless  cells  up  to  several  hun- 
dreds of  thousands  per  c.mm.,  the  proportion  of  the  white  to  the  red 
often  being  1:  20,  less  frequently  1:  10  to  1:1.  There  is  a  considerable 
increase  of  mononuclear  cells  of  the  lymphatic  type  (see  Plate  7,  Fig. 
5),  which  however  may  be  of  varying  size,  and  especially  in  the  acute 
form  often  attains  to  a  considerable  size  (large  lymphocytes,  Ehrhch, 
medullary  cells  of  several  authors,  not  to  be  confounded  with  Ehrlich's 
granulated  myelocytes;  central  germ  cells,  Benda);  sometimes  they  are 
exceedingly  friable.  Polynuclear  cells  in  these  cases  amount  only  to  a  few 
per  cent.  Often  there  is  nuclear  segmentation.  In  myelogenous  leukae- 
mia (mixed  celled,  see  Plate  7,  Fig.  4)  there  are  aside  from  considerable 
augmentation  of  the  polynuclear  cells: 

1.  Mononuclear,  neutrophile  or  eosinophile  cells  (Ehrlich's  myelo- 
cytes) which  do  not  exist  in  normal  blood. 

2.  Absolute  and  relative  augmentation  of  the  "mastzellen"  (poly- 
nuclear cells  with  basophile  granulation). 

3.  Atypical  cell  forms  (karyokinesis,  extremely  small  or  large 
forms,  polynuclear  cells  with  granulation  sUght  or  absent). 


DISEASES    OF    THE    BLOOD  161 

The  red  blood  coi-puscles  are  nearly  always  decreased,  perhaps  to 
2  or  3  milUons,  and  seldom  are  the  values  below.  Nummular  forma- 
tion is  nearly  always  absent,  there  are  polychromatic  and  granular 
degenerations,  nucleated  cells  (mostly  normoblasts),  less  often  poikilo- 
cytes,  microcytes  and  megalocytes.  In  myelogenous  leuka-mia  there 
are  mast  cells  also  in  the  exudates. 

The  glands,  which  are  nearly  always  palpable,  do  not  as  a  rule  ex- 
ceed the  size  of  a  hazel-nut,  although  at  autopsy  more  exten.sive  enlarge- 
ments are  often  disclosed  than  were  at  first  supposed.  Also  the  other 
lymphatic  formations,  especially  in  the  fauces,  are  oedematous,  and  here 
there  are  frequent  ulcerations  with  consequent  liEemorrhages  and  sep- 
tic conditions,  often  there  is  an  ulcerous  stomatitis.  The  spleen  is  like- 
wise axlematous,  but  does  not  usually  attain  to  a  very  considerable  size 
except  in  chronic  leukiemia.  The  hver  is  often  enlarged.  Hsmorrhages 
which  often  defy  control  are  visible  in  the  skin  and  mucous  membranes. 
The  exudates  are  usually  of  a  sanguineous  coloration.  Retinal  ha'mor- 
rhages  are  hardly  ever  absent  in  acute  leukaemia,  sometimes  there  is  a 
leukffimic  retinitis  with  white  foci.  It  is  quite  usual  that  very  large 
quantities  of  uric  acid  are  excreted  with  the  urine  (Virchow,  A.  FrJinkel, 
Magnus-Levy). 

The  course  may  be  very  acute  (death  after  3  weeks),  but  many  cases 
drag  on  for  many  (4J)  years.  In  these  cases  there  are  temporary  peri- 
ods of  improvement  rn  the  blood  and  general  conditions.  Septic  infec- 
tions may  lead  to  a  chsappearance  of  the  leuka-mic  blood  picture  and 
decrease  of  the  swelhngs  through  a  destruction  of  the  cells,  according  to 
A.  Frankel  (aleukaemic  stage). 

The  diagnosis  is  based  upon  (1)  the  blood  findings  (very  high  poly- 
nuclear  leucocytosis  alone  proves  nothing);  (2)  enlargement  of  the 
organs;  (3)  hsemorrhagic  diathesis  (retinal  hsemorrhages).  In  atypical 
cases  the  differential  diagnosis  may  be  very  difficult  as  against  perni- 
cious ana-mia  (Geissler  and  Japha,  Ai-neth-Leube).  The  difficulties  in 
young  children  where  there  is  already  a  relative  lymphocytosis,  and 
where  there  are  also  myelocytes,  have  already  been  dealt  ■with  (p.  160). 

Therapy. — Operative  interference  (extirpation  of  lymph-nodes  and 
spleen)  has  only  an  injurious  effect.  In  view  of  the  peculiar  effect  of 
infectious  diseases  upon  the  blood  picture,  remedies  have  been  adminis- 
tered for  their  chemotactic  effect  (extract  of  spleen,  spermin,  tuberculin, 
nuclein,  cinnamic  acid),  but  all  without  success.  Temporary  success 
may  follow  after  iron,  arsenic,  iodine,  the  latter  being  also  used  exter- 
nally: quinine  and  phosphorus  have  been  less  successful.  Attempts 
have  been  made  to  influence  the  spleen  by  ergotin  injections,  the  appli- 
cation of  the  icebag,  also  in  adults  by  berberinum  sulf.,  three  times  daily 
0.01-0.03  Gm.  (J-J  gr.),  dyspnoea  by  inhalations  of  oxygen.  'Move  recently 
the   X-ray  treatment  has  attracted  attention  in  chronic   cases  which 

11—11 


16i2 


THE   DISEASES   OF   CHILDREN 


were  not  yet  complicated  by  grave  anemia.  Considerable  improvement 
has  thereby  been  achieved  and,  although  only  in  very  few  cases,  also 
maintained  for  several  years  after  discontinuance  of  treatment.  The 
variation  in  success  is  perhaps  explained  to  a  certain  extent  by  the 
treatment  which  varied  according  to  site  (spleen,  bones,  glands,  Uver) 
and  the  duration  of  exposure  (daily  or  weekly,  or  a  totahty  varying  be- 
tween fifty  and  sixty  thousand 
minutes).  The  undeniable  effect 
is  explained  by  most  authors  (de 
la  Camp)  by  the  specific  influence 
of  the  X-rays  on  the  lymphoid 
tissue,  by  Arneth  by  their  influ- 
ence on  the  circulating  blood  (the 
supposed  micro-organisms). 

PSEUDOLEUK.ffi;MIA 

Nature,  Name  and  Forms. — 

Pseudoleuka>mia  .simulates  leukse- 
inia  by  its  chnical  course,  the  ap- 
pearance of  the  patient,  and  the 
anatomical  findings  (hyperplasia 
of  all  lymphatic  formations),  al- 
though the  principal  .sign,  the 
"white  blood  corpuscles,"  is  miss- 
ing. The  name  given  to  this  affec- 
tion by  Cohnheim  in  1865  chiefly 
expresses  sometlung  negative,  and 
similarly  the  various  other  names 
under  which  similar  symptom  com- 
plexes are  grouped  (adenie,  Trous- 
seau; lymphosarcoma,  Virchow, 
Langhans;  malignant  lymphoma, 
Billroth ;  lymphatic  or  splenic  anae- 
mia, Griesinger,  Striimpell)  mark 
the  uncertainty  of  the  chnical 
picture.  The  designation  of  Hodg- 
kin's  disease  is  explained  by  the  fact  that  Hodgkin  in  1832  pubhshed 
observations  on  hyperplasia  of  glands  and  spleen  which  produced  gen- 
eral manifestations;  leukaemia  as  such  was  not  yet  discovered  at  that  time 
and  his  observations  therefore  hardly  referred  to  a  uniform  affection. 

Like  leuksemia,  pseudoleukemia  was  also  formerly  differentiated  as 
pseudoleuka-inia  lymphatica  and  Uenalis,  according  to  the  chief  enlarge- 
ment, to  which  was  added  pseudoleukemia  medullaris  later  on,  but 
according  to  more  recent  investigations  the  primary  origination  from 


Chronic  leuksemia.    Boy  from  Fig.  23.    .^terior 
view  of  spleen. 


DISEASES    OF     THE    HLOOI) 


163 


Fig.  25 


the  glands  is  decidedly  the  most  frequent;  exclusive  localization  in  the 
spleen  is  rare,  while  an  affection  of  the  medulla  leads  in  most  instances  to 
leukiemia  (Neumann,  Grawitz).  Pappenlieim  is  of  the  opinion  that  the 
same  irritation  produces  pseudoleuka?mia  and  leuka-mia  according  to 
whether  it  affects  onlj-  the  lymphatic  glands  and  spleen  or  also  the 
medulla;  similarly  Pincus  places  tiie  affection  in  close  relation  to  leu- 
kaemia, principally  based  upon  a 
relative  lymphocytosis  whicli  he 
has  always  found  in  true  pseudo- 
leukiiemia.  Gra^^^tz,  on  the  other 
hand,  was  unable  to  verify  this 
observation  by  the  material  at  liis 
disposal.  After  some  previous  in- 
vestigators (Askanazi,  Weishaupt, 
Brentano  and  Tangl)  had  discov- 
ered tubercle  baciUi  in  pseudo- 
leuka?mic  swellings,  C.  Sternberg 
pubUshed  the  remarkable  fact  that 
15  out  of  18  closely  observed  clini- 
cal cases  were  founded  on  tuber- 
culosis, in  which  the  microscope 
revealed  a  difference  froiy  true 
lymphomata  by  the  existence  of 
special  cells  and  caseation,  with- 
out the  necessity  of  the  presence 
of  the  typical  Ijlood  picture. 
While  admitting  that  tubercle 
bacilU  may  be  found  accidentally 
in  pseudoleuksemic  glands,  yet  the 
great  frequency  of  the  findings 
justifies  the  conclusion  that  the 
major  part  of  the  observed  cases 
of  pseudoleukaemia  are  attribu- 
table to  a  tuberculous  affection  of 
the  lymphatic  structures  (also  in 
animal   experiments    tuberculosis 

may  cause  lymphomata  of  simple  appearance).  Grawitz,  however,  states 
that  a  difference  between  these  cases  and  true  pseudoleukiemia  cannot 
be  cUnically  established,  that  both  groups  may  be  equally  influenced  by 
arsenic  and  he  thus  makes  the  etiology  the  basis  of  a  classification  In- 
differentiating  pseudoleukccmias  of  a  simply  lymphomatous  (better  per- 
haps "lymphomatosis  of  unknown  origin"),  tuljerculous  and  sypliilitic 
origin.  The  common  symptom  is  the  clinical  jiicture:  the  progressive 
involvement  of  all  the  lymphatic  organs.     According  to  many  observa- 


Clirouic  leukaemia.    I.ati  ra 
maten^lb 


164  THE    DISEASES   OF   CHILDREN 

tions  formation  of  lymphomata  follows  in  the  wake  of  an  inflammatory 
process  in  the  root  area  of  the  lymphatic  glands  (gastric  affections, 
chronic  coryza,  defective  teeth,  inflammations  of  the  ear),  supposedly 
also  of  malaria,  whooping-cough,  dysentery.  Reported  cases  of  family 
affections  do  not  stand  criticism. 

Occurrence. — Youthful  individuals  are  chiefly  affected,  and  the 
period  of  childhood  is  considerably  involved.  Fischer  had  7  children 
among  12  patients,  Meyer  among  76  cases,  11  up  to  10  years  and  7  up 
to  20  years  of  age;  Falkenthal  out  of  40  cases  8  in  the  first  and  11  in  the 
second  decade.    It  is  not  known  in  how  far  the  tuberculous  form  prevails. 

Anatomy. — At  autopsy  an  extensive  involvement  of  the  lymphatic 
system,  also  foci  in  the  internal  organs,  are  disclosed  as  in  leuktemia, 
but  often  still  more  extensive  than  in  the  latter.  The  simple  lymphomata 
behave  under  the  microscope  precisely  as  those  of  lymphatic  leuksemia. 
In  the  cases  of  tuberculous  involvement  Sternberg  found  singularly  large 
mononuclear  or  polynuclear  cells  with  abundant  protoplasm  and  large 
nuclei,  often  with  nucleated  corpuscles,  also  karyokinesis.  Frequently 
there  are  necrotic  foci  which  do  not  occur  in  true  lymphoma;  tubercle 
bacilli  and  typical  tuberculous  tissue  are  not  seen  in  all  cases.  The 
medulla  has  not  frequently  been  included  in  the  field  of  examination. 
More  frequently  amyloid  degeneration  has  been  observed,  sometimes 
also  true  tuberculous  affections  of  internal  qrgans  (Fischer). 

Symptoms  and  Course. — The  course  is  such  that  a  group  of  lym- 
phatic glands,  most  frequently  cervical,  swell  on  one  or  both  sides  of  the 
neck,  growing  to  lobular  tumors  sometimes  of  a  larger  size  than  the  fist 
of  a  man.  Sometimes  the  lymphatic  structures  of  fauces,  tongue  and 
tonsils  are  involved  from  the  first.  Then  proliferation  begins  in  a  neigh- 
boring glandular  group  or  on  the  opposite  side.  One  gland  after  another, 
corresponding  to  the  lymphatic  current,  is  involved  in  the  proliferation, 
until  the  new  formation  reaches  into  the  cavities  of  the  body.  Then 
there  are  metastases  in  the  remote  glands,  in  the  internal  organs,  fol- 
lowed by  enlargement  of  liver  and  spleen.  The  spleen  may  reach  an 
enormous  size;  sometimes  there  is  tenderness  in  the  bones.  The  numer- 
ous swelhngs  frequently  lead  to  manifestations  of  compression,  pressure 
in  the  vessels  leads  to  engorgement  in  the  upper  or  lower  half  of  the  body, 
pressure  on  the  trachea  and  oesophagus  causes  difficulty  in  respiration 
and  swallowing.  Finally  cachexia  and  ana^nia  develop.  The  blood 
shows  a  diminution  of  hemoglobin  and  red  blood  corpuscles,  but  grave 
changes  in  the  erythrocytes  are  less  frequent.  The  white  cells  are 
often  moderately  increased.  An  increase  of  lymphocytes  (Pincus)  only 
occurs  in  simple  lymphomata,  but  according  to  Grawitz  is  not  always 
present,  while  in  cases  with  a  tuberculous  foundation  there  is  usually 
polynuclear  leucocytosis.  The  skin  is  more  frequently  involved,  there 
are  pruritic  affections,  subcutaneous  lymphomata,  also  purpura.     The 


DISEASES    OF   THE    BLOOD 


165 


r 


i 


urine,  on  the  whole,  does  not  present  any  definitely  established  pecu- 
liarities; sometimes  there  is  albumin,  especially  when  there  is  amyloid 
degeneration  which  occurs  rather  frequently.  Fever  is  one  of  the  usual 
manifestations  and  may  be  very  liigh.  Ebstein  thinks  he  can  establish 
a  special  type  as  that  of  chronic  relapsing  fever,  in  which  febrile  and 
afebrile  periods  alternate. 

The  duration  of  the  affection  may  occupy  years,  exacerbations  may 
alternate  with  improvements  of  the  general  condition  and  reduction  of 
the  glands;  intercurrent  diseases  p^^  .,,, 

(varicella,  measles)  sometimes 
effect  a  transitory  improvement 
(Bendix).  In  the  end  death 
usually  ensues  from  cachexia, 
amyloid  degeneration,  septic 
affections,  pneumonia. 

The  prognosis  is  always 
doubtful,  and  although  a  cure 
is  not  absolutely  excluded  (Kat- 
zenstein,  Grawitz),  relapses  may 
at  any  time  be  expected.  In 
some  cases  the  therapy  may  be 
able  to  postpone  the  unfavorable 
termination  for  years. 

The  diagnosis  cannot  be 
established  mth  certainty  at 
the  onset  of  the  disease,  later 
this  is  easier.  Scrofulosis  gen- 
erally does  not  produce  glandu- 
lar enlargements  of  such  mag- 
nitude; in  pseudoleuktemia,  too, 
the  other  scrofulous  symptoms 
are  absent,  such  as  blepharitis, 
coryza,  phlycta?nular  affections 

of  the  cornea,  aural  secretions.  Lymphoma  on  a  tuberculous  founda- 
tion (Sternberg)  may  be  distinguished  from  the  simple  lymphoma  at 
autopsy  only.  Differentiation  from  leuka:'mia  is  only  possible  by  the 
examination  of  the  blood.  There  is  a  peculiar  difficulty  in  the  first 
stage  of  the  disease  by  the  presence  of  lymphosarcomatous  affections 
(Kundrat).  At  first  they  commence  precisely  Hke  pseudoleuktcmic 
swellings,  but  then  they  break  through  the  glandular  capsules  and  lead 
to  adhesions  of  the  glands  between  each  other  and  their  surroundings; 
besides,  ulcerations  of  the  buccal  mucous  membrane  and  the  upper 
air  tracts  may  develop.  The  recognition  of  solitary  splenic  enlarge- 
ments is  often  difficult  (see  also  ]).   166). 


L_ 


P^eudoleukiPmia.  Six-year-old  boy  with  hiird,  loii- 
ular,  glandular  tumor,  the  size  of  a  child 'ti;  head,  a 
smaller  glandular  tumor  in  the  axilla.  Distinct  manifes?- 
tation.s  of  engorgement  in  the  left  half  of  the  face.  The 
glandular  tumor  of  the  neck  will  be  extirpated.  There  are 
glands  without  caseation  and  true  tuberculous  changes. 
Final  outcome  uncertain. 


1(>() 


THE   DISEASES   OF   CHILDREN 


In  regard  to  prophylaxis  some  authors  recommend  the  extirpation 
of  glanduhir  enlargements  of  long  standing  which  resist  other  treatment. 
Otherwise  the  remarks  made  for  leukaemia  apply  here  hkewise,  includ- 
ing operative  therapy  and  X-ray  treatment  which — at  least  temporarily 
— may  without  doubt  have  a  favorable  influence  on  the  enlargements 
and  the  general  conditions.  The  only  point  to  be  added  is  that  intelli- 
gent  treatment    with   arsenic   is   attended   with   undeniable  success,   if 


Fig.  27. 
Relations  of  Figures  in  Nokmal  and  Pathological  Blood 


=-       '^  Minimum     J 


i  Minimum 
■  Maxitimm 


of  Erythro- 
cytes 


Neuirophile 
I'olynuclear  cells 


Lymphocytes 


Myelocytea 


[  Mononuclear  leucocytes 
I     antl  transition  forms 


|i|;;:-:      :;;;|  Eosinophile  cells 
I  Mast  cells 


New-   InfantJr  Adults  Pseudo-   Perni-  Lymphatic  Myelogenous 
born  leukre-      cious      leukEemia      leukaemia 

mia       anupmia 
in  adults 

Tlie  left  columns,  hatched  traversely,  give  the  minimum  and  maximum  of  the  red  blood  corpuscles,  the 
riuht  ones  give  the  proportion  of  tlie  colorless  corpuscles.  For  instance  the  diagram  shows  at  once  the  large 
number  of  lymphatic  cells  in  pseudoleukjemic  aniemia  and  their  almost  total  disappearance  in  myeloid  leuk:smia. 

applied  in  accordance  with  the   maxims  laid  down  on  p.   139.     Even 
complete  recoveries  have  been  reported  which  had  not  too  far  advanced 

(Grawitz,  Katzenstein). 


AFFECTIONS  OF  THE  SPLEEN 

Tlie  spleen  becomes  enlarged  in  many  infectious  diseases,  as  in 
typlioid,  i)aralysis  of  recurrent  laryngeal  nerves,  malaria,  tuberculous 
meningitis,  parotitis.  Considerable  enlargements  occur  in  leukemia, 
pseudoleuka^mia,  and  brief  mention  may  be  made  of  echinococcus  of 
the  spleen. 


DISEASES   OF   THE    BLOOD  167 

Of  rather  frequent  occurrence  is  anomaly  of  position,  splenic  ptosis. 
It  is  found  even  in  infants  and  is  nearly  always  associated  with  a  general 
flabbiness  of  the  entire  musculature,  especially  that  of  the  abdomen, 
often  also  with  a  diastasis  of  the  recti  muscles.  In  older  children  there 
is  usually  a  general  enteroptosis  including  the  downward  displacement 
of  liver  and  kidneys.  Generally  these  children  are  languid,  of  marked 
nervous  irritability  and  small  appetite.  In  these  cases  the  complaints 
depend  not  so  much  on  the  displacement  as  on  the  nerves,  and  only 
occasionally  has  a  disturbance  originating  with  the  spleen  (kinking  of 
the  intestine)  been  observed  in  adults.  The  diagnosis  is  estabhshed 
from  the  general  condition;  again,  the  prolapsed  spleen  is  much  softer 
than  one  really  enlarged.  The  therapy  endeavors  to  improve  the  ner- 
vous complaints,  the  general  circulation,  to  effect  a  sHght  fattening,  and 
is  therefore  to  follow  the  same  directions  as  in  infantile  anaemia  where  a 
similar  enteroptosis  is  occasionally  observed. 

The  so-called  rachitic  enlargement  of  the  spleen  is  not  a  typical 
accompaniment  of  rachitis,  as  has  been  stated  by  Henoch.  Sasuchin 
found  splenic  enlargement  12  times  in  66  cases,  Geissler  and  Japha  22 
times  in  75,  Cohn  only  58  times  in  858  cases.  Sometimes  it  is  only 
simulated  by  prolapse  mth  engorgement,  and  for  this  reason  a  spleen 
wliich  was  distinctly  palpable  during  hfe,  is  sometimes  not  found  en- 
larged at  autopsy.  Real  enlargement,  however,  may  sometimes  have  a 
connection  \vith  a  coexisting  ana?mia.  Enlargement  of  the  spleen  is 
probably  not  caused  by  rachitis,  but  at  the  most  by  a  factor  similar  to 
the  one  wMch  caused  the  latter. 

Syphihtic  splenic  enlargement  sometimes  occurs  quite  indepen- 
dently. In  congenitally  syphilitic  infants  it  is  not  found  so  frequently 
as  is  sometimes  stated.  On  the  other  hand  there  are  sometimes  very 
considerable  enlargements  in  older  children  with  congenital  syphilis, 
although  recent  sypliihtic  manifestations  need  not  be  present.  Some- 
times the  diagnosis  can  be  established  only  from  the  history,  in  other 
cases  the  condition  is  indicated  by  infantiUsm  and  Hutcliinson's  teeth. 
Usually  there  is  also  an  enlargement  of  the  liver,  in  rare  cases  syphilis 
of  bones  and  joints.  These  manifestations  are  difficult  to  treat  even  by 
energetic  antisypMlitic  therapy. 

The  so-called  Banti's  disease  (anaemia  accompanied  by  spleno- 
megaly) begins,  according  to  this  author's  description,  \\"ith  enlarge- 
ment of  the  spleen  which,  in  conjunction  with  anaemia,  may  in  the  course 
of  years  assume  considerable  proportions.  The  annemic  stage  ha\ing 
lasted  for  several  }'ears  is  complicated  by  ascites  and  hepatic  cirrhosis, 
and  the  patient  dies  from  haemorrhages  under  dropsical  manifestations 
(Senator).  The  blood  findings  are  characterized  by  considerable  oligo- 
chromjemia,  then  oUgocythsemia,  also  leukopenia,  with  a  preponderance 
of  lymphocytes.    Banti  attributes  the  affection  to  an  intoxicating  process 


168  THE    DISEASES   OF    CHILDREN 

emanating  from  the  spleen,  and  indeed  some  authors  (Maragliano, 
Bessel-Hagen)  report  having  effected  cm-es  by  extirpating  the  spleen. 
The  affection  has  often  been  observed  in  children  (Osier  11-year-old 
girl,  Morse  7-year-old  boy,  Senator  15-year-old  boy,  Pribram  15-year- 
old  boy).  ^The  diagnosis  can  only  be  established  after  exclusion  of  all 
other  causes  of  enlarged  spleen.  The  only  cui'e  is  held  to  be  the  ex- 
tirpation of  the  spleen,  while  leukaemia  is  not  influenced  by  tliis  opera- 
tion. Latterly,  Chiari,  Marchand,  looked  to  late  hereditary  syphihs  for 
the  cause  of  similar  cases,  because  a  distinct  lobulated  Uver  was  discov- 
ered at  autopsy.  According  to  this  finding  the  liver  must  be  at  least 
involved  simultaneously  with  the  spleen,  and  the  extirpation  of  the  spleen 
would  simply  act  hke  Talma's  operation  on  the  existing  ascites.  If 
these  reports  should  be  verified  for  at  least  part  of  the  cases,  the  therapy 
ought  to  be  antisyphihtic;  the  effect,  however,  can  only  be  conditional, 
because  the  pathological  process  has  already  partly  run  its  course  by 
the  time  it  comes    under  observation. 

AFFECTIONS  OF  THE  BONE  MARROW 

Red  marrow  fills  the  medullary  spaces  of  the  short  and  flat  bones 
(sternum,  ribs,  vertebrse,  cranial  bones)  and  also  the  diaphyses  of  the 
long  tubular  bones  in  the  foetus.  Soon  after  birth  the  red  marrow 
begins  to  be  replaced  by  adipose  marrow,  the  transformation  being  com- 
plete in  the  diaphyses  of  the  tubular  bones.  The  red  marrow,  however, 
soon  returns  when  an  irritation  is  exercised  by  certain  diseases  (infec- 
tions) or  ha?morrhages.  The  "red  marrow"  of  pernicious  antemia 
differs  from  the  normal  red  marrow;  according  to  Ehrlich  it  is  megalo- 
blastic, and  the  leukaemic  marrow,  too,  deviates  from  the  normal. 
Latterly  the  bone  marrow  has  been  credited  the  role  of  storing  up 
protective  substances  in  infectious  diseases. 

In  adults  a  tumefaction  has  several  times  been  observed  in  the 
medulla,  the  so-called  myeloma  (Mahler,  Senator).  It  consists  of  lym- 
phoid cells  or  cells  of  the  medullary  type.  It  chiefly  affects  the  ribs  and 
short  bones,  later  also  all  the  other  bones.  As  a  sequel  grave  anaemia 
occurs  of  the  pernicious  type.  The  urine  contains  Bence-Jones'  albumi- 
noid bodies.     No  cases  have  thus  far  been  observed  in  children. 


HiEMORRHAGIC  AFFECTIONS 

uy 
Dr.  R.  HECKER,  of  Munich 

translated  by 
Dr.  EDWARD  F.  WOOD,  Wilmington,  N.  C. 


Under  the  name  of  Hsemorrhagic  Affections  or  Haemorrhagic  Diath- 
esis a  number  of  pathological  conditions  are  grouped  together  which 
have  in  common  a  fundamental  tendency  to  blood  extravasations. 
These  affections  occur  apparently  more  or  less  independently  and  are 
therefore  to  be  differentiated  from  the  secondary  extravasations  which 
occur  as  a  sequel  to  other  diseases.  This  line,  however,  cannot  always 
be  drawn  sharply,  there  being  rather  a  large  number  of  transition  cases 
which  are  difficult  to  group.  Secondary  haemorrhages  are  for  instance 
observed  in  infectious  diseases,  especially  diphtheria,  hereditary  syphi- 
lis, septic  processes,  leukaemia,  etc. 

The  tendency  to  haemorrhages  may  be  congenital  and  in  that  case 
habitual,  a  condition  known  by  the  name  of  haemophilia,  or  it  may  only 
temporarily  be  present  in  particular  individuals. 

This  latter  group  of  transitory  haemorrhagic  diatheses  is  repre- 
sented by  the  various  forms  of  purpuric  affections:  purpura  simplex, 
purpura  rheumatica,  purpura  lurmorrhagica,  purpura  abdominalis,  pur- 
pura fulminans,  further  by  scorbutus,  infantile  scurvy,  melama  neona- 
torum and  paroxysmal  haemoglobinuria. 

Except  for  om-  increased  knowledge  of  infantile  scurvy  (which  has 
been  treated  in  a  different  place)  the  last  twenty-five  years  have  not 
wrought  any  very  startling  changes  in  our  knowledge  of  haemorrhagic 
affections,  since  the  epoch-making  works  of  Immermann,  Grandidier 
and  Henoch.  True,  bacteriological  investigations  have  here,  too,  exer- 
cised a  stimulating  effect,  the  more  so  as  many  of  the  observed  purpura 
cases  gave  rise  to  the  supposition  that  infectious  processes  were  at  the 
bottom  of  them.  The  search  for  a  specific  cause  of  purpura  haemor- 
rhagica  kept  many  investigators  busy,  but  the  results  of  the  excellent 
work  of  Letzerich,  Babes,  Finkelstein,  von  Dungern,  and  others,  are  not 
yet  satisfactory.  Moreover  the  opinion  represented  chiefly  by  Giroux 
and  Cattaneo  has  gained  ground,  namely  that  purpura  is  not  an  indepen- 
dent affection  at  all,  but  merely  a  symptom-complex  caused  b}^  a  num- 
ber of  different  factors. 

The  old  doctrine  of  Immermann  regarding  the  diminished  coagula- 
bility of  the  blood  has  experienced  scientific  examination  and  partial 

169 


170  .    THE   DISEASES   OF   CHILDREN 

confirmation  by  the  recent  investigations  of  Sahli.  Therapeutically 
progress  was  made  by  the  introduction  of  gelatin  on  the  recommenda- 
tion of  Zuppinger  and  Baginsky;  of  adrenal  preparations  to  control 
haemorrhage;  of  atropine,  to  check  impending  intestinal  symptoms 
(Hecker);  and  also  by  the  precise  study  of  ha>mophile  articular  ha'mor- 
rhages  by  Konig  wliich  helped  to  put  this  affection,  which  has  long 
been  mistaken  as  "rheumatic,"  into  its  right  place. 

HEMOPHILIA 

Bleeders'  disease,  or  the  habitual  tendency  to  haemorrhages  is  in 
most  cases  congenital.    It  is  considered  the  most  hereditary  of  all  diseases. 

There  are  two  types  of  "bleeders"  according  to  whether  the  haemor- 
rhages occur  after  a  trauma  or  apparently  without  cause:  transitory 
bleeders  or  spontaneous  bleeders.  A  sharp  Une  of  demarcation  cannot 
be  drawn,  it  being  accepted  as  probable  that  even  the  second  class  owes 
its  haemorrhages  to  small  traumata  which  had  escaped  attention. 

Occurrence. — The  first  manifestations  occur  in  most  cases  (about  65 
per  cent.)  in  infants  under  2  years  of  age,  very  rarely  in  persons  over  22 
years  of  age.  In  infancy  traumatic  haemorrhages  are  generally  more 
frequent  than  spontaneous  ones.  The  affection  has  a  predilection  for 
temperate  latitudes,  and  Germany  furnishes  the  largest  number  of 
cases.  Boys  have  a  much  greater  tendency  to  it  than  girls,  according  to 
Grandidier  13  times  and  to  von  Ethnger  Si  times  as  great. 

Heredity. — Although  heredity  in  many  patients  cannot  be  demon- 
strated, in  most  of  them  the  affection  can  be  traced  through  several 
generations.  In  many  famihes  the  ancestral  taint  has  been  present  for 
over  a  century.  For  tliis  reason  there  are  usually  a  number  of  bleeders 
in  one  family,  while  sporadic  cases  are  rare.  While  the  male  members  of 
a  family  are  affected  much  more  frequently  than  the  female  ones,  the 
latter  have  the  peculiar  abihty  of  transmitting  the  affection  to  their 
offspring  without  ever  having  been  bleeders  themselves.  Hgemophihc 
men  generally  procreate  healthy  children  through  Avomen  who  have  not 
sprung  from  bleeder  families,  whereas  hsemophilic  women  as  a  rule  pro- 
duce cliildren  with  the  affection.  Non-ha?mophihc  male  members  of 
bleeder  fariiilies  hardly  ever  procreate  hirmophihc  children.  Exceptions 
however  are  known  where  the  female  members  were  particularly  affected. 

Some  indi-\aduals  or  whole  families  display  under  certain  circum- 
stances a  tendency 'to  only  spontaneous  or  only  traumatic  hemorrhages. 

Symptoms. — The  long-continued  existence  and  the  difficulty  of  con- 
trolling these  haemorrhages  is  more  characteristic  than  their  severity, 
which  is  often  not  at  all  great.  The  haemorrhage  may  last  for  days, 
weeks  or  months,  the  blood  not  spurting,  but  slowly  oozing  from  the 
capillaries. 

The  traumatic  haemorrhages  may  occur  into  the  interstitial  tissue, 


H.«MORRHAGIC   AFFECTIONS  171 

skin,  muscles  or  articulations,  where  under  certain  circumstances  they 
maj'  assume  the  shape  of  large  extravasations;  or  they  may  appear  on 
the  free  surface  of  the  skin  or  mucous  memhianes  on  the  slightest  provo- 
cation; irregularly  torn  wounds  are  more  apt  to  give  rise  to  obstinate 
haemorrhages  than  smooth  cut  ones.  Ritual  circumcision  seems  to  be 
especially  fraught  with  danger  in  this  regard;  hasmorrhage  of  this  kind 
has  also  been  observed  after  paracentesis  of  the  tympanic  membrane 
(Thompson).     Vaccination  has  given  little  cause  for  apprehension. 

Spontaneous  haemorrhages  are  sometimes  preceded  by  prodroniata 
in  the  shape  of  vertigo,  lassitude,  sensation  of  cold  or  perspiration. 
The  haemorrhages  occur  either  on  the  free  surfaces  of  mucous  membranes 
or  into  the  subcutaneous  cellular  tissue.  Among  the  former  bleeding 
from  the  nose  and  the  buccal  cavity  (on  the  eruption  of  the  teeth)  is 
freciuent  and  specially  dangerous.  Bleeding  from  the  umbilicus  is  sur- 
prisingly rare  in  bleeder  families.  The  interstitial  haemorrhages  of  the 
skin,  generally  a  very  early  and  very  regular  symptom  of  htemophilia, 
sometimes  appear  in  the  shape  of  petechia?,  sometimes  of  regular  tu- 
mors (hsematoma)  in  the  subcutaneous  cellular  tissue  (Neter).  They  are 
usually  situated  at  the  lower  extremities  and  the  lower  part  of  the  trunk. 
Their  color  differs  according  to  age  of  the  haemorrhages  from  dark  and 
bluish  red  to  brown  and  light  yellow.  As  a  rule  the  petechiae  appear  in 
crops,  disappearing  again  completely  by  absorption.  Fairly  large  blood 
extravasations  may  however  cause  suppuration  which  it  is  dangerous 
to  incise  as  this  may  give  rise  to  fresh  Ijleeding  and  sepsis. 

Complications. — Frequently  in  the  course  of  haemophilia  there  are 
haemorrhages  into  the  skin  which  indeed  may  be  the  only  symptom; 
there  are  colorless,  soft,  elastic  enlargements,  generally  at  the  knee  and 
hip-joints,  wliich  were  formerly  regarded  by  patients  and  physicians 
ahke  as  rheumatic  affections.  Even  if  these  articular  haemorrhages 
usually  terminate  favorably  by  resorption,  it  may  well  happen  that  the 
simple  haemarthritic  effusion  changes  to  chronic  panarthritis  and  its 
sequelffi — serious  deformations  and  contractures  (Konig). 

Stiffness  of  the  linibs  may  also  arise  from  extensive  interstitial 
haemorrhages,  wMch  by  connective  tissue  organization  may  lead  to 
atrophy  of  muscles  and  tendons. 

Etiology.— Practically  nothing  is  known  in  regard  to  the  causation 
of  hicmophilia.  This  is  sufficiently  proven  by  the  large  number  of  hy- 
potheses, none  of  which  is  supported  by  demonstrable  facts.  Lossen  and 
Grandidier  assumed  a  faulty  mixture  of  the  blood,  causing  impaired 
coagulability;  Immermann  assumed  an  augmented  total  quantity  of 
blood,  or  pathological  plethora;  Virchow,  congenital  stenosis  of  the 
arteries  and  thinness  of  their  walls;  Alierhalden,  an  anatomical  anomaly 
in  the  construction  of  the  vessels  differing  according  to  localization:  W. 
Koch,  an  infectious  cause;  SahH,  a  disturbed  chemism  of  the  vascular 


1>^  THE   DISEASES   OF   CHILDREN 

walls  which  being  transmitted  with  the  germ  plasma  would  lead  to  ab- 
normal friability  and  permeability.  What  has  really  been  demonstrated 
in  a  few  cases  is  fatty  degeneration  of  the  vascular  intima,  and  enlarged 
endotheha  with  swelling  of  the  nuclei  (Virchow,  Hooper,  Linton). 

The  examination  of  the  blood  showed  different  degrees  of  diminished 
haemoglobin,  and  microscopically  the  picture  of  severe  anaemia  with 
poikilocytosis  and  nm;leated  erythrocytes  (Faludi).  SahU  has  latterly 
demonstrated  that  the  polynuclear  leucocytes  are  reduced  both  abso- 
lutely and  relatively  and  that  the  quantity  of  fibrin  and  the  physical 
properties  of  the  blood  are  normal.  The  time  of  coagulation  of  the  blood 
was  normal  at  the  time  of  the  lupmorrhages,  or  possibly  somewhat 
prolonged,  wliile  at  the  time  when  there  were  no  haemorrhages  it  was 
considerably  protracted. 

Gangrenous  processes  may  be  caused  by  the  pressure  of  an  extrav- 
asation in  the  interstitial  tissue  upon  the  neighboring  parts.  This 
causes  atrophy  either  of  small  principal  parts,  or,  when  larger  vessels 
are  being  compressed,  of  entire  extremities. 

Course  and  Prognosis. — A  particular  hsemorrhage  may  be  checked 
either  by  artificial  aid,  or  spontaneously  by  lowering  the  blood  pressure. 
If  the  haemorrhages  continue  for  a  long  time,  the  blood  becomes  con- 
stantly lighter  and  thinner  and  death  may  supervene  with  manifesta- 
tions of  extreme  anaemia.  In  other  cases,  however,  the  patient  may 
rapidly  recover  in  a  relatively  short  time  from  even  very  severe  blood 
losses,  and  just  this  tolerance  of  bleeders  in  the  face  of  profuse  blood 
losses  is  remarkable.  A  large  proportion  of  ha?mophilic  patients,  how- 
ever, succumb  early  in  childhood  owing  to  internal  and  external  haemor- 
rhages, 60  per  cent,  dying  before  the  eighth  year  and  only  11  per  cent. 
reaching  the  eleventh  year  (Litten).  The  total  mortality  of  htemophiles 
is  87  per  cent,  (von  Ethnger). 

THE  PURPURA  AFFECTIONS 

These  are  a  series  of  disease  pictures  which  have  the  one  point  in 
common  that,  in  the  shape  of  an  independent  disease,  transitory  and 
spontaneous  haemorrhages  occur  in  internal  and  external  parts;  a  tran- 
sitory ha?morrhagic  diathesis. 

For  a  long  time  various  forms  of  purpura  have  been  distinguished; 
the  recognition,  however,  has  gradually  gained  ground  that  they  all 
present  the  same  jjathological  picture,  differing  merely  in  degree  and 
locahzatiou:  haemorrhages  of  purpura  simplex  merely  on  the  external 
skin;  of  purpura  rheumatica,  skin  haemorrhages  \\ith  simultaneous 
involvement  of  the  articulations;  of  purpura  abdominahs,  skin  haemor- 
rhages with  severe  gastric  and  intestinal  manifestations,  perhaps  also 
articular  affections;  of  purpura  fulminans,  the  gravest  form  of  skin 
haemorrhages;  of  purpura  haemorrhagica,  or  morbus  maculosis  Werlhoffii, 


H.EMORRHAGIC    AFFECTIONS  173 

skin  haemorrhages  combined  with  haemorrhages  of  the  mucous  mem- 
branes or  into  internal  organs.  All  these  forms  cannot  be  strictly 
separated,  chnicall}',  etiologically  or  anatomically.  One  form  may  co- 
exist WTth  another  or  change  into  it.  From  this  point  of  view  tlie 
divisions  used  in  the  following  pages  should  be  understood. 

Occurrence. — Purpura  is  rather  a  rare  disease,  principally  affecting 
children  after  the  third  year;  infants  are  almost  entirely  spared.  For- 
ster  observed  an  average  of  1  case  in  every  1300  out-patients.  Girls 
seem  to  be  somewhat  more  predisposed  to  it  than  boys;  there  are  more 
cases  in  -ndnter  than  in  summer.  According  to  C.  Koch's  statements 
concerning  the  frequency  of  purpura  in  St.  Petersburg,  the  influence  of 
some  local  tendency  may  be  supposed  to  be  at  work. 

Etiology. — Although  certain  factors  must  certainly  be  considered 
as  indirect  causes,  such  as  weak  constitution,  chronic  aiitemia,  clu'onic 
intestinal  catarrh,  unhealthy  dwelhngs,  malnutrition  especially  by 
starch  and  preserved  food,  a  direct  cause  cannot  be  stated  at  the  present 
time.  With  great  probability  and  in  some  cases  with  certainty  it  may 
be  assumed  that  infectious  processes  are  present.  The  attempts,  how- 
ever, to  point  to  a  specific  causative  factor  have  so  far  been  doomed  to 
failure  and  will  probably  so  continue,  it  having  been  found  that  bac- 
teria of  different  types  may  under  certain  circumstances  produce  hipmor- 
rhagic  affections.  Successful  experiments  have  been  made  to  obtain 
pure  cultures  of  various  bacteria  from  both  the  maculse  and  the  blood 
of  purpura  patients  and  to  reproduce  ha?morrhagic  diseases  therewith  in 
animals,  for  instance  streptococci  (Hochheimer),  staphylococci  ("staph, 
cereus  albus"  Fiorentini),  colon-like  rods  (Benedetti),  bacillus  pyo- 
cyaneus  (Neumann,  Hecker),  bacillus  purpurse  (Letzerich,  Gimard,  Kolb, 
Babes),  capsule  diplococci  (von  Dungern),  and  a  bacillus  resembling 
that  of  mouse  septicsemia  (Finkelstein).  Anything  specific  however  can 
probably  not  be  attributed  to  any  of  these  bacteria.  Typical  purpura 
has  been  observed  in  the  course  of,  and  following  leuktemia,  diphtheria, 
and  especially  of  angina  lacunaris  (Bruck).  The  opinion  held  by  Giroux 
and  Cattaneo  that  purpura  is  not  an  independent  disease  but  a  symp- 
tom-complex, continues  to  gain  ground,  and  it  is  quite  possible  that 
such  symptom-complex  may  also  be  produced  by  non-bacterial  causes 
such  as  chills,  alcohoUsm,  overexertion,  although  even  in  these  cases  a 
latent  infection  cannot  be  excluded. 

The  manner  of  the  occurrence  of  these  haemorrhages  is  equally  ob- 
scure. In  cases  where  bacterial  embolisms  are  present — and  these  form 
the  majority — we  must  not  content  ourselves  ■nith  the  assumption  of  a 
toxic  influence  upon  the  capillary  walls  which  favors  a  diapedesis  of  the 
red  blood.  Diminished  coagulability  of  the  fibrin  which  had  formerly 
been  supposed  to  exist,  cannot  be  demonstrated  either  chemically  or 
microscopically. 


174 


THE    DISEASES   OF   CHILDREN 


Anatomy. — Accordingly,  the  anatomical  yield  is  small.  In  grave 
cases  of  purpura  ha>morrhagica  there  are  parenchymatous  degenerations 
of  heart  and  liver,  also  hypoplasia  of  the  bone  marrow  (Muir). 

In  milder  cases  the  blood  shows  a  diminution  of  the  wliite  and  red 
K„,..>,s.  blood    corpuscles,    of 

the  htemoglobin  and 
of  the  specific  grav- 
ity; in  grave  cases 
(jnu-pura  haemorrha- 
gica)  considerable  leu- 
cocytosis,  poikilocy- 
tosis  and  microblasts. 

PURPURA  SIMPLEX 

Purpura  simplex 
is  characterized  by 
shght  blood  extrava- 
sations into  the  skin 
only.  These  appear 
q  u  i  t  e  unexpectedly, 
or  they  are  antici- 
pated by  certain  pre- 
cursors several  days 
in  advance,  such  as 
headache,  anorexia, 
lassitude,  vomiting. 
The  maculae  have  the 
size  of  a  pinhead  to 
a  lentil,  are  of  circu- 
lar shape,  resembhng 
fleabites  or  weals; 
they  are  isolated  and 
do  not  coalesce.  The 
fresh  maculae  are  dark 
red  or  bluish  red,  af- 
Purpura  simplex.  tcr    some   time   they 

become  paler  and  pass 
through  the  various  stages  of  the  blood-pigment.  In  some  of  them  there 
is  a  palpable  indurated  spot  in  the  centre  (fibrinous  coagulation).  They  do 
not  disappear  on  pressure.  In  their  distribution  they  show  a  predilection 
for  certain  parts  of  the  body;  thus  the  face  and  usually  the  hands  also 
are  left  free,  wliile  a  preference  is  shown  for  the  extensor  surfaces  of  the 
lower  extremities  and  the  arms;  as  they  extend  further,  they  invade  also 
the  trunk.    The  eruption  occurs  either  all  at  once  or  advances  by  jumps. 


H,EMORRHAGlC    AFFECTIONS  175 

In  the  latter  case  macules  of  every  age  may  be  noticed  side  by  side. 

The  general  condition  is  usually  not  disturbed  at  all;  prodromal 
manifestations,  should  such  exist,  usually  disappear  with  the  eruption. 
Fever,  as  a  rule,  is  absent. 

There  are  sometimes  de\'iations  in  the  exanthem  to  such  an  extent 
that  the  petechiae  are  infiltrated  like  oedematous  wheals — purpura  urti- 
cans (Biedert).  Further,  a  true  urticaria  or  exudative  eczema  may 
develop  side  by  side  with  the  raacuhu  of  the  purpura. 

Course  and  Termination. — The  duration  of  the  pathological  process 
depends  upon  whether  the  petechise  appear  practically  at  the  same  time 
or  at  more  or  less  distant  intervals.  In  the  former  case  eight  to  twelve 
days  are  reckoned  to  effect  a  cure,  in  the  latter  case  it  may  require  weeks 
or  months.  The  termination  is  always  favorable;  only  where  there  is 
considerable  extension  of  haemorrhage  and  a  prolonged  duration  of  the 
affection,  there  may  be  manifestations  of  ansemia. 

PURPURA  RHEUMATICA.     PELIOSIS  RHEUIVIATICA 

(Schonlpin) 

T^lien  purpura  simplex  is  comphcated  by  articular  pains  and  swell- 
ings wliich  dominate  the  disease  picture,  it  is  termed  purpura  rheumatica. 

Symptoms. — The  affection  frequently  commences  ^\-ith  certain 
prodromal  signs:  lassitude,  anorexia,  also  vomiting  or  diarrhoea,  vague 
pains  in  the  hmbs  which  are  not  yet  localized  at  the  joints,  sometimes 
urticarial  eruptions.  Then  fever  sets  in,  followed  by  the  appearance  of 
the  red  macules.  The  latter  are  usually  a  little  larger  than  in  purpura 
simplex,  do  not  coalesce,  and  are  generally  found  only  on  the  legs  up  to 
just  above  the  knee,  but  may  also  appear  in  other  places.  They  are 
always  most  numerous  in  the  neighborhuod  of  the  joints  of  the  extrem- 
ities. Sometimes  they  protrude  above  the  level  of  the  skin,  the  extrav- 
asation on  account  of  its  hardness  being  palpable. 

The  articular  pains  and  swellings  appear  either  before  or  after  the 
eruption,  the  joints  of  the  feet  and  knees  being  affected  most  frequently. 
The  oedema  is  caused  by  serous  infiltration  of  the  periarticular  parts. 
Thus  there  is  no  question  of  articular  inflammation  as  in  articular 
rheumatism,  or  of  haemorrhages  into  the  joints  as  in  ha^mophihc 
articular  affections.  Both  the  absence  of  any  cardiac  involvement 
and  the  regidarly  favorable  termination  of  these  articular  affections 
form  further  differentiating  points  as  against  the  other  two  affections 
named. 

Frequently  not  only  the  joints,  but  also  the  bones  of  the  lower  ex- 
tremities are  painful  on  pressure. 

A  pecuhar  point,  also  shared  with  purpura  simplex,  is  the  not 
infrequent  combination  of  the  petechia  ^^^th  other  skin  affections,  such 
as  erythema  nodosum,   multiform  exudative  erythema,  urticaria,  etc., 


176 


THE    DISEASES   OF   CHILDREN 


so  that  the  nodules  of  erythema  nodosum  may,  for  example,  change  to 
blood  extravasations  or  the  urticaria  wheals  may  be  filled  with  blood 
(Neter). 

In  the  course  of  the  disease  there  are  frequent  collections  of  oedema, 
especially  at  the  lower  extremities,  the  scrotum,  elbows  and  eyelids, 
although  no  albumin  is  demonstrable  in  the  urine. 

The  general  condition  is  but  shghtly  disturbed  on  the  whole,  Init 
elevations  of  temperature  up  to  40°  C.   (104°  F.)  have  been  observed. 


Purpura  rheumatica.  Girl  aged  one  year  and  nine  months,  .\cute  onset  with 
articular  swelling  and  skin  hapraorrhages  at  the  trunk  and  extremities.  Spots  up  to 
lentil  size  and  especially  numerou.s  at  the  ti?dematous  joints.  The  a-dema  around  the 
ankle-joints  is  continued  to  the  root  of  the  toes. 


Course  and  Termination. — In  acute  cases  with  rapid  onset  the  affec- 
tion lasts  about  fourteen  days,  usually  however  it  takes  a  paroxysmal 
course,  so  that  a  few  days  after  the  disappearance  of  the  manifestations 
there  appear  fresh  eruptions  with  renewed  fever  and  other  articular 
swellings.  These  relapses  are  especially  encouraged  by  patients  leaving 
the  bed  too  soon.    The  termination  is  always  favorable. 


H.EMORRHAGIC    AFFECTIONS 


177 


Fig.  30. 


PURPURA  ILEMORRHAOICA.    MORBUS  MACULOSUS 
WERLHOFII 

The  haemorrhages  occur  not  only  on  the  external  skin  and  in  the 
subcutaneous  cellular  tissue,  but  also  in  various  mucous  membranes 
and  in  the  internal  organs. 

The  affection  usually 
begins  quite  suddenly 
without  prodromata  while 
patients  are  in  the  best  of 
health,  by  the  appearance 
of  blood  spots  over  the  en- 
tire trunk  and  the  extremi- 
ties. These  spots  are  partly 
similar  to  tho.se  in  purpura 
simplex,  but  most  of  them 
are  considerably  larger  up 
to  the  size  of  a  small  dish 
and  coalesce  into  large 
patches.  Their  contour  is 
irregular,  partly  round,  part- 
ly oval  and  partly  striated; 
color  dark  red  with  bluish 
or  brownish  tint;  and  the 
body  attains  quite  a  pecu- 
liar tiger-like  appearance. 
Here  and  there  the  hsemor- 
rhages  assume  the  form  of 
subcutaneous  infiltrations. 
In  severe  cases,  where  the 
blood  spots  coalesce  to  a 
considerable  extent,  thepar- 
ticular  extremity  appears 
quite  dark,  cedematous,  and 
covered  with  wheals  similar 
to  gangrene,  except  that 
the  odor  is  ab.sent.  Some- 
times the  haemorrhages 
extend  over  small  areas, 
but  invade  deeper  layers 
and    form    coarse     knots. 

These  skin  haemorrhages 
are  a.ssociated  with  hirmorrhages  from  all  kinds  of  mucous  membranes, 
especially  from  the  no.=;e.     Epistaxis  is  one  of  the  regular  symptoms  of 
the  affection.     Then  there  appear  blood  spots  on  the  mucous  membranes 
11—12 


Purpura  li.Tmorrlmsrica.  Kieht-year-old  girl;  acute  attack 
witli  fever.  Dark,  bluish  reil  blooil  spots  as  large  as  half  a 
dollar  to  a  small  plate  in  the  skin  of  (he  upper  and  lower  ex- 
tremities. The  malar  mucous  membrane  likewise  shows  small 
punctiform  ha-morrhages.     Cure  after  S'o  weeks. 


178  THE    DISEASES   OF   CHILDREN 

of  the  lips,  palate  and  tongue,  less  frequently  on  the  conjunctiva  or  in 
the  ear.  Hirmorrhages  on  the  mucous  membranes  of  the  intestine  and 
the  bladder  are  shown  by  the  excretion  of  bloody  stools  and  bloody  urine, 
but,  like  hsemoptosis  and  hsematemesis,  this  occurs  only  in  very  rare  and 
very  severe  cases.     The  joints,  as  a  rule,  remain  uninvolved. 

There  is  such  a  pronounced  general  tendency  to  hajmorrhage  that 
sUght  pressure  on  any  part  of  the  body  suffices  to  produce  an  extrava- 
sation of  blood  into  the  skin,  the  subcutaneous  cellular  tissue,  or  the 
joints.  In  .slight  external  injuries  occasioned  by  scratching  with  the 
fingernails,  injections,  punctures  in  blood  examinations,  there  are  often 
hsemorrhages  which  may  become  dangerous  on  account  of  the  difficulty 
to  control  them. 

The  general  condition  is  sometimes  disturbed  only  .slightly  but  in 
many  cases  very  perceptibly:  the  children  are  ill-humored,  fagged  out, 
tired,  ask  to  go  to  bed,  complain  of  headache  and  look  pale.  In  .severe 
cases  an  almost  typhoid  condition  may  be  developed.  The  temperature 
is  not  materially  elevated  as  a  rule,  but  under  certain  circumstances 
may  rise  to  39.5°  C.  (103°  F.)  in  the  evening.  Pulse  sometimes  slow.  More 
serious  disturbances  of  the  general  condition  will  then  appear,  if  severe 
and  frequent  epistaxis  has  caused  profuse  loss  of  blood.  The  debility 
may  then  become  pronounced. 

Course  and  Termination. — The  majority  of  cases  have  an  acute 
course  without  any  actual  repetition  of  the  attacks.  When  the  spots 
have  reached  the  cUmax  in  point  of  number  and  extent,  which  is  gen- 
erally the  case  in  about  a  week,  they  become  paler  and  change  color 
along  with  the  changes  of  the  blood-pigment.  The  frequent  bleedings 
from  nose  and  mouth  come  to  a  standstill,  feces  or  urine  which  may 
have  been  tinged  with  blood,  resume  normal  conditions  and  after  about 
fourteen  days  recovery  is  complete. 

Sometimes,  however,  the  onset  is  slow,  and  then  the  affection  takes 
a  much  more  chronic  course.  The  hiEmorrhages  on  the  skin,  from  the 
nose  and  gums,  etc.,  are  so  frequently  repeated  that  many  weeks  and 
months  may  elapse  before  a  cure  is  effected.  In  fact,  when  the  inter- 
vals of  apparent  health  are  of  longer  duration,  the  trouble  extends  over 
several  years.  These  are  the  cases  which  owing  to  considerable  loss  of 
blood  may  lead  to  grave  conditions  and  even  death.  On  the  other  hand, 
cases  have  been  reported  which  in  spite  of  an  acute  course  have  ended 
fatally  within  a  few  days.  On  the  whole,  however,  the  termination  is 
favorable. 

ABDOMINAL     PURPURA     (Henoch) 

In  the  course  of  a  rheumatic  purpura  abdominal  manifestations, 
such  as  vomiting,  intestinal  hsemorrhages  and  colic  may  appear  under 
certain   circumstances.      These  are  productive  of  a  peculiar  symptom- 


H/EMORRHAGIC    AFFECTIONS  179 

complex  which  Henoch  observed  in  several  cases  in  1868  and  described 
in  1874.  Since  then  a  number  of  these  cases  have  been  published.  The 
course  is  generally  as  follows:  Sometimes  after  macules  and  (x'dema 
have  e.xisted  in  various  joints,  certain  dyspeptic  complaints  occur, 
the  articular  pains  become  more  severe,  and  new  blood  extravasations 
make  their  appearance.  Vomiting  is  exceedingly  obstinate  and  diffi- 
cult to  control,  the  vomited  matter  con.sisting  of  colorless  or  greenish 
mucus  at  first,  and  changing  later  to  dark  bloody  masses.  Attacks  of 
violent  colicky  pains  torment  the  patient  to  such  an  extent  that  he 
groans  and  cries  out  in  his  bed.  The  pains  generally  increase  until  a 
defecation  has  taken  place,  which  is  generally  accompanied  with  con- 
siderable tenesmus.  The  stools  at  first  scant  and  hard,  become  diar- 
rha>al,  assuming  a  blackish,  dark  red  or  orange  yellow  color.  Anorexia 
is  complete.  In  consequence  of  the  pains,  vomiting  and  loss  of  blood, 
patients  become  rapidly  debilitated  and  give  the  impression  of  being 
seriously  ill. 

The  objective  .signs  may  be  multiform  small  and  mcilium-sized 
petechiae,  oedema,  painfulness  and  stiffness  of  the  knee  and  ankle-joints, 
sometimes  also  of  the  elbow-joints.  The  articular  regions  are  hkewise 
the  seat  of  the  densest  macular  eruptions.  The  abdomen  is  distended 
and  usually  highly  sen.sitive  to  pressure  in  the  region  of  the  transverse 
colon.  There  is  fever,  which  however  does  not  exceed  38.5°  C.  (101°  F.) 
as  a  rule.  The  buccal  ca\'ity  remains  free  from  luemorrhages;  there  are 
no  cardiac  changes. 

Like  all  forms  of  purpura,  the  abdominal  variety  is  particularly 
characterized  by  paroxysmal  manifestations  with  intervals  of  days, 
weeks  or  even  a  year,  which  tend  to  protract  the  illness  considerably. 
The  attacks  themselves  gradually  diminish  in  vehemence,  or  the 
relapses  may  concern  only  the  blood  spots  or  only  the  articular 
swelling.  Aside  from  these  fully  developed  cases  there  are  others 
in  which  one  or  other  of  the  symptoms  is  absent,  for  instance  the 
articular  swelhng. 

Henoch's  purpura,  hke  all  other  forms  of  purpura,  should  not  be 
treated  as  an  affection  sui  generis,  there  being  only  a  question  of  specific 
localization  of  the  affection  in  the  area  of  the  intestinal  tract.  No  ana- 
tomical observations  in  children  liave  been  reported,  but  the  assump- 
tion of  blood  extravasation  into  the  mucous  membranes  of  the  stomach 
and  intestine  will  probably  not  be  far  wrong.  It  is  an  undecided  point 
as  to  what  makes  the  intestine  so  sensitive.  In  the  case  of  a  ten-year- 
old  boy  observed  by  the  author  the  habitual  con.sumption  of  alcohol 
(son  of  a  restaurant  keeper)  and  marked  errors  of  diet  were  held  respon- 
sible for  the  cause  of  the  first  attack  and  the  following  relapses. 

The  prognosis  is  always  to  be  made  with  caution  on  account  of  the 
grave  condition  and  the  impending  danger  of  nephritis. 


180  THE   DISEASES   OF   CHILDREN 

PURPURA  FULMINANS 
This  affection,  which  was  Hkewise  first  described  by  Henoch,  rep- 
resents an  exceedingly  rare,  but  the  gravest,  modification  o.f  purpura 
simplex.  While  haemorrhages  from  mucous  membranes  are  absent,  ex- 
tensive ecchymoses  develop  with  alarming  rapidity.  They  appear  bilat- 
erally and  rather  symmetrically,  discoloring  entire  extremities  ^\^tllin  a 
few  hours,  first  bluish  red,  then  blue  and  black-red,  and  causing  a  coarse 
blood  infiltration  of  the  cutis.  There  is  often  a  formation  of  serosan- 
guineous  vesicles  upon  the  skin,  but  never  gangrene,  nor  is  there  any 
fetid  odor.  The  course  is  alarmingly  rapid  and  always  fatal;  within 
12-24  hours  from  the  formation  of  the  first  blood  spot  death  supervenes; 
the  longest  period  was  four  days.  There  are  no  comphcations,  autopsy 
yielding  a  negative  result  with  the  exception  of  general  ana?mia.  In  a  few 
cases  there  are  reports  of  a  history  of  preceding  acute  infectious  diseases, 
in  others  however  there  was  a  total  absence  of  etiological  indications. 

SCORBUTUS 

Scurvy  is  a  transitory  hsemorrhagic  diathesis  wliich  is  associated 
with  severe  disturbance  of  nutrition,  and  with  a  tendency  to  ulceration 
and  ichorization.  In  cliildhood  it  certainly  occurs  rather  rarely.  M61- 
ler-Barlow's  disease  which  by  many  is  termed  infantile  scurvy,  and  true 
scorbutus  should  be  considered  distinct. 

Etiologically  there  may  possibly  be  certain  infectious  causes  such  as 
streptococci  and  staphylococci,  but  the  essential  condition  is  a  body  pre- 
pared for  the  development  of  scurvy  by  improper  nutrition  and  unhy- 
gienic conditions.  Food  poor  in  vegetable  acid  alkahes  is  held  especially 
responsible  (Immermann) :  long-continued  nutrition  with  flom'-foods,  con- 
densed, preserved  or  sterilized  milk,  inferior  bread,  want  of  fresh  vegetables, 
fruit,  fresh  meat.  A  further  necessity  for  the  development  of  the  affec- 
tion seems  to  be  continued  Hving  in  dark,  ill-hghted,  damp  dwellings. 

Symptoms. — The  affection  never  begins  suddenly,  but  always 
slowly,  exliibiting  signs  of  gradually  advancing  cachexia,  emaciation, 
pallor  of  the  skin  and  mucous  membranes,  disturbances  in  the  cardiac 
and  intestinal  functions.  To  tliis  is  added  a  specific  scorbutic  affection 
of  the  gums;  extensive  painful  swelUng,  and  loosening  of  the  gums, 
wliich  bleed  at  every  touch,  also  loosening  of  the  teeth.  Frequently 
there  is  necrotic  disintegration  of  the  marginal  parts,  which  become 
desquamated  and  form  a  slate-colored,  ulcerating  gray  surface. 

To  complete  the  pathological  picture,  there  are  numerous  petechise 
and  ecchymoses  into  the  skin,  the  connective  tissue  and  muscles,  on 
mucous  and  serous  membranes,  in  the  periosteum  and  on  the  retina. 
There  is  also  actual  bleeding,  especially  from  the  nose;  feces  and  urine 
tinged  with  blood  are  less  frequent.  Enlargement  of  the  spleen  may 
also  develop. 


H.EMORRHAGIC    AFFECTIONS  181 

Blood  Findings. — Examination  of  tlie  blood  does  not  disclose  any- 
thing reail}-  cliaracteri.stic.  Corresponding  to  the  losses  of  blood  there 
is  a  diminution  of  htemoglobin  and  red  blood  corpuscles.  Haj^em, 
Robin  and  Pentzold  observed  small  cori)uscles  resembUng  blood  plate- 
lets of  strong  refractive  power. 

Course  and  Termination. — .Scorbutus  always  takes  a  chronic  pro- 
tracted course,  but  tliere  is  no  accentuation  of  paro.xysmal  attacks. 
Mild  cases  may  be  cured,  severe  ones  frequently  terminate  fatally,  as 
a  rule  in  consequence  of  complications,  ulcerations,  septic  processes, 
pleuritis,  pericarditis.     The  prognosis  is  therefore  doubtful. 

PAROXYSMAL   H^EMOGLOBINURIA 

Haemoglobinuria  from  cokl;  Psychogemc  Htemoglobinuria. 

In  this  affection  there  are  paroxysmal  secretions  of  dark  blood- 
colored  urine,  ^\ith  or  without  ascertained  causes.  It  should  be  dis- 
tinguished from  hsemoglobinuria  of  the  newborn  (Winckel's  disease) 
and  from  symptomatic  hiemoglobinuria  which  occurs  after  burns,  poi- 
soning with  phosphorus,  chlorate  of  potash,  mushrooms,  and  has  no 
paroxysmal  character. 

Symptoms. — The  attack  is  usually  preceded  by  a  state  of  general 
irritability,  lassitude,  yaw-ning;  the  attack  itself  sets  in  with  chills, 
sensation  of  great  cold,  cyanosis,  promptly  followed  by  a  state  of  heat 
and  perspiration.  Sometimes  there  is  even  collapse.  Then  there  is  a 
secretion  of  blood-colored  urine,  at  first  usually  accompanied  by  severe 
pains.  Frequently  there  are  hyper £emic  spots  appearing  simultaneously 
on  the  skin,  especially  in  parts  affected  by  the  cold,  sometimes  there  are 
wheals.  A  few  patients  exliibit  under  certain  circumstances  gangrene 
at  various  parts  of  the  body. 

The  urine  is  either  blackish,  dark  red.  burgundy  or  claret  colored, 
but  always  dark  colored.  It  contains  abundant  albumin,  gives  Heller's 
and  .\lmen"s  blood  test,  but  in  the  microscopic  picture  blood  corpuscles 
are  absent.  On  the  other  hand,  there  are  brownish,  lumpy  masses.  In 
the  spectrum  it  shows  the  bands  of  metha^moglobin. 

The  blood  in  the  first  paroxysm  shows  hipmoglobintemia,  the  serum 
containing  haemoglobin;  there  are  also  pale  erythrocytes  and  so-called 
shadows  (Burkhard).  After  the  paroxysm  both  htemoglobin  and  red 
blood  corpuscles  are  diminished.  The  blood,  however,  recuperates  very 
rapidly,  so  that  on  the  following  day  the  examination  shows  the  blood 
already  normal.  During  the  interval  between  paroxysms  there  are 
never  traces  of  htemoglobin  in  the  blood  serum. 

Etiology. — As  a  predisposing  factor  there  is  at  the  bottom  of  many 
cases  a  pre^ious  chronic  or  acute  infectious  disease,  especially  hered- 
itary syphihs,  malaria,  scarlet  fever,  and  as  immediate  cause  there  is 
almost  always  a  severe  chill  or  thorough  wetting;  hence  the  appellation 


182  THE   DISEASES    OF    CHILDREN 

cold  hsemoglobinuria.  Infectious  factors  do  not  appear  to  have  any 
influence,  this  being  probably  a  neurosis  which  chiefly  affects  the 
vasomotor  system  (von  Reckhnghausen). 

Probably  the  chill  causes  primarily  a  change  in  the  chemico-biolog- 
ical  composition  of  the  plasma  enabhng  it  to  exert  a  hipmolytic  influ- 
ence upon  the  blood  corpuscles.  Especially  suitable  to  the  production 
of  hsemolysis  is  cold  in  conjunction  with  congestion.  It  is  possible  to 
produce  by  artificial  experimentation  hitmoglobina>mia  and  in  specially 
predisposed  persons  also  hajmoglobinuria,  by  cutting  off  the  blood  supply 
of  a  finger  and  after  a  while  dipping  the  finger  into  cold  water  (Ehrlich's 
experiment),  or  by  giving  the  patient  a  cold  foot  bath. 

Course  and  Prognosis. — A  single  paroxysm  generally  lasts  I2  to  2 
hours.  The  paroxysms  are  repeated  in  irregular  intervals  according  to 
the  possibihty  of  exposure  to  cold,  and  they  are  more  frequent  in  winter 
than  in  summer.  The  prognosis  depends  upon  the  nature  of  the  original 
trouble,  but  is  on  the  whole  favorable. 

DIAGNOSIS  FOR  HiEMORRHAGIC  AFFECTIONS 

The  recognition  of  fairly  pronounced  cases  is  easy.  The  strict 
diagnostic  separation  of  the  various  forms  of  purpura  is  without  practi- 
cal importance;  in  case  of  need  a  review  of  the  points  mentioned  on 
page  172  in  regard  to  the  uniformity  of  the  various  forms  of  purpura 
ought  to  be  sufficient.  An  early  recognition  of  heemophiha  would 
be  important,  as  the  life  of  the  patient  may  thereby  be  prolonged  for 
years;  but  unless  there  is  a  bleeder  family  in  the  case,  the  diagnosis 
is  difficult  and  probably  only  possible  after  the  first  serious  haMnor- 
rhage.  Frequent  recurrence  of  "rheumatic"  pains  in  limbs  and  joints 
requires  careful  observation  if  it  occurs  in  a  member  of  a  bleeder 
family,  as  the  pains  may  exist  for  a  long  time  as  the  only  expression  of 
a  latent  haemophilic  diathesis.  Considering  that  these  articular  affec- 
tions represent  so  to  speak  a  noli  me  tangere,  it  is  necessary  to  differen- 
tiate them  from  other  similar  affections.  They  are  most  easily  confused 
with  tuberculous  wliite  swelhng,  from  which  they  may  be  distinguished 
by  the  rapid  appearance  and  disappearance  of  the  exudates  and  by 
the  absence  of  any  considerable  thickening  of  the  capsule. 

Haemophihc  articular  affections  as  well  as  articular  swelhngs  in 
rheumatic  purpura  are  distinguished  from  articular  rheumatism  by  the 
larger  swelling  in  the  latter,  the  local  development  of  heat,  the  moist 
skin  tending  to  perspiration  and  fever.  It  should  be  understood  that  in 
hiemophiha  there  are  hfpmorrhages  into  the  joints,  that  in  purpura 
there  is  oedematous  swelhng  of  the  periarticular  parts,  that  in  rheuma- 
tism there  is  inflammatory  swelhng  and  effusion  into  the  joints  and 
their  neighborhood,  that  in  tuberculous  arthritis  there  is  granulation 
which  always  considerably  involves  the  adjacent  bones.     In  all  these 


H.EMORRHAGIC    AFFECTIONS  183 

cases  X-ray  examination  will  prove  a  most   excellent  aid  in  diagnosis. 

Morbus  Maculosus  and  Scorbutus. — These  two  affections  are  dif- 
ferent in  their  very  onset.  In  the  former  it  is  more  or  less  sudden,  in 
the  latter  after  slow  preparation  of  the  soil.  Although  in  the  course  of 
purpura  hemorrhagica  a  severe  disturbance  of  nutrition  may  set  in,  it 
is  always  a  secondary  occurrence  and  never  present  .simultaneously  with 
the  first  appearance  of  the  other  manifestations;  such  however  is  the 
rase  in  scorbutus,  in  which  along  with  early  disturlied  nutrition,  there 
is  a  characteristic  tendency  to  ulceration  and  inflammation.  The  af- 
fected gums  in  scorbutus  are  known  by  their  dark  red  swelling,  their 
spongy  con.sistency,  the  loosening  and  sensitiveness  of  the  gums,  all 
manifestations  which  are  absent  in  purpura. 

The  urine  in  hcematuria  is  distinct  from  that  in  hcBtnoglobinuria  by 
its  lake-colored  appearance  and  the  percentage  of  the  red  blood  corpus- 
cles; in  the  latter  di.sease  attention  .should  be  paid  to  the  paroxysmal 
occurrence  in  conjunction  with  the  effect  of  cold.  Hiematm'ia  occm"s, 
aside  from  haemorrhagic  diatheses,  when  there  are  stones  in  the  bladder, 
the  renal  pelvis  or  the  kidney,  a  condition  generally  associated  with 
considerable   secretion   of   mucous   and  inflammatory   products  in   the 

urine. 

TREATMENT  OF  H.ffi;MORRHAGIC  AFFECTIONS 

Haemophilia. — Correct  projjhylaxis  should  endeavor  to  restrict  the 
procreation  of  htemopliihc  children.  According  to  Grandidier's  ex- 
perience it  is  well  to  discountenance  marriage  of  female  members  of 
bleeder  famiUes,  whether  they  themselves  are  bleeders  or  not;  male 
members,  however,  unless  they  are  bleeders  themselves,  may  be  per- 
mitted to  marry.  Male  bleeders  should  only  then  be  dissuaded  from 
marrying  if  there  is  proof  that  in  their  families  hsemophilic  men  have 
procreated  hiemophilic  cMldren,  always  provided  that  the  males  in 
question  had  married  healthy  daughters  of  healthy  families. 

Individual  prophylaxis  should  commence  immediately  after  birth, 
and  in  view  of  the  dangerous  character  of  the  htemorrhages  be  carried 
through  with  persistency  during  the  first  few  years  of  life.  Every  injury, 
be  it  ever  so  .slight,  should  be  prevented;  for  tliis  reason  all  surgical 
interference  is  contraindicated,  as  for  instance  operation  for  cleft  palate, 
removal  of  ntevi,  piercing  of  earlobes,  and  particularly  circumcision. 
Vaccination,  however,  has  always  proved  free  from  danger.  Taking 
great  care  of  the  buccal  cavity,  preventing  as  far  as  possible  the  ex- 
traction of  teeth,  and  selecting  toys,  furniture  and  articles  for  domestic 
use  with  circumspection,  are  important.  In  later  Ufe  caution  at  begin- 
ning of  menstruation,  interdiction  of  gymnastic  exercises,  selection  of  a 
suitable  vocation,  are  points  commanding  attention. 

The  general  treatment  should  endeavor  to  strengthen  the  entire 
organism,  for  wliich  purpose  a  mild  diet  with  plenty  of  fresh  vegetables 


184  THE    DISEASES   OF   CHILDREN 

and  salad  should  be  prescribed,  avoiding  articles  which  excite  the  vascu- 
lar system,  such  as  alcohol,  coft'ee,  tea.  Cold  friction,  saline  baths,  resi- 
dence in  the  country  or  at  the  seaside,  are  efficacious  adjuvants. 

Internally,  vegetable  acids  (lemon  cure)  may  certainly  be  tried, 
although  the  success  is  doubtful.  The  same  apphes  to  the  administra- 
tion of  sahne  remedies  or  the  reduction  in  the  supply  of  fluids  recom- 
mended by  Immermann  and  Oertel  on  account  of  plethora  wliich  they 
suppose  to  exist. 

The  special  treatment  of  the  ha3morrhages  consists  in  the  first  place 
in  elevating  the  affected  part  of  the  body,  wliich  is  often  sufficient.  In 
the  second  place  apply  compression,  tamponade,  the  cautery,  com- 
pression or  ligation  of  the  nearest  vascular  trunks.  For  a  local  hemo- 
static the  custom  now  is  to  apply,  aside  from  chloride  of  iron,  the 
adrenal  preparations:  adrenalin  or  suprarenin  in  solutions  of  I  :  1000. 
Hesse  recommends  a  solution  of  calcium  chloride.  Good  results  have 
been  obtained  by  gelatin  treatment,  injecting  25  Gm.  (3vi)  of  Merck's 
10  per  cent.  "Gelatina  SteriUsata  pro  Injectione." 

The  treatment  of  hsemophiUc  articular  affections  consists  in  rest 
and  moderate  compression;  when  the  pains  are  severe  apply  moist 
packing,  from  the  second  or  third  day  massage  of  the  centrally  situated 
parts.  As  to  operative  interference  nothing  but  aseptic  puncture  is 
admissible.     Later  on  orthopedic  measures  may  become  necessary. 

Purpura. — In  view  of  the  uncertain  etiology  of  purpura  there  can 
be  no  question  of  causal  treatment.    The  foremost  measure  is  thorough. 

General  Treatment. — In  all  cases,  even  the  mildest,  strict  rest  in 
bed  is  necessary.  But  this  very  requirement  frequently  meets  with 
great  objection,  because  the  patients,  enjoying  otherwise  good  health, 
can  be  kept  in  bed  only  with  difficulty,  wliile  parents  are  not  easily  con- 
vinced of  the  necessity  of  the  measure.  It  should  be  remembered  that 
the  frequency  of  relapses  is  usually  due  to  failure  to  observe  these  in- 
structions. The  sick  room  should  be  well  ventilated  and  kept  cool.  The 
diet  should  be  bland,  not  seasoned;  all  exciting  substances, — alcohol, 
coffee,  tea, — should  be  avoided  and  the  preference  given  to  milk,  Hght 
farinaceous  dishes  and  vegetables.  Large  meals  are  injurious;  instead, 
small  portions  should  be  given  every  two  or  three  hours.  Constipation, 
which  may  readily  occur,  should  be  overcome  by  the  use  of  grated 
apples,  senna-infusion,  castor  oil.  Highly  indicated  are  regular  baths,  to 
wWch  decoctions  of  oak  bark  and  nut  leaves  have  been  added,  as  they 
contribute  to  the  more  rapid  resorption  of  skin  haemorrhages.  In  very 
protracted  cases  a  change  of  chmate  is  often  useful.  As  an  after-cure, 
a  general  strengthening  of  the  body  is  necessary  and  a  stay  in  the  moun- 
tains or  at  the  sea-side  advisable.  Special  attention  is  frequently  de- 
manded by  secondary  anaemia,  the  treatment  of  which  is  to  be  conducted 
according  to  the  usual  rules. 


H.EMORUHAGIC    AFFECTIONS  18.5 

The  special  treatment  is  purely  symptomatic.  The  ha>morrhages 
cease  of  their  own  accord  with  qiiiet  rest  in  bed.  In  epistaxis  prescribe 
horizontal  position  with  lowered  head,  compression  of  the  affected  ala, 
sniffing  up  cold  water  in  which  a  few  drops  of  chloride  of  iron  solution 
has  been  mixed,  and  tamponade.  In  internal  haemorrhages,  no  time 
should  be  wasted  over  ergotin,  which  is  uncertain  in  action;  there  should 
be  immediate  and  repeated  injections  of  20-.30  c.c.  (ov-.5i)  of  a  10  per 
cent,  gelatin  solution;  for  internal  administration  up  to  200  Gm.  f.5vi) 
daily  of  the  same  solution  may  be  given.  Treatment  of  the  abdominal 
symptoms,  such  as  occur  in  mild  degrees  in  morbus  maculosus,  and  in 
the  gravest  degree  in  Henoch's  purpura,  demands  special  attention. 
Here  absolute  rest,  application  of  the  icebag  on  the  abdomen  and  strict- 
est diet  are  in  order.  Cooled  milk,  cold  albumin  water,  cold  almond 
milk,  should  be  given  by  the  teaspoonful,  until  the  stormy  manifestations 
have  come  to  an  end.  Intestinal  haemorrhages  should  be  checked  by 
a  diluted  solution  of  iron  chloride  given  by  the  teaspoonful,  gelatin 
subcutaneously  or  internally.  For  the  pain  give  opium.  The  success  of 
these  remedies,  however,  is  by  no  means  positive.  In  a  grave  case  in 
my  practice  atropine  rendered  excellent  service.  The  pains  as  well  as 
the  hiemorrhages  ceased  instantly  after  an  injection  of  0.0003  Gm.  (-r^gr.) 
atropine  sulphate.  The  remedy  has  not  only  an  instantly  antispasmodic 
action,  but  evidently  an  ischipmic  effect  upon  the  intestinal  vessels. 
Rectal  irrigations  mth  a  1  per  cent,  solution  of  lead  or  aluminum  acetate 
are  recommended. 

Scorbutus. — By  way  of  prophylaxis  infants  should  be  fed  as  long  as 
pos.sible  on  mother's  milk;  otherwise  fresh,  raw  or  recently  boiled  milk 
should  be  given,  also  fruit  juice.  Older  children  receive  raw  milk,  plenty 
of  fresh  vegetables,  fruit  and  salad.  Generally  speaking,  an  improve- 
ment of  the  hygienic  conditions  should  be  attempted.  These  measures 
are  also  applicable  to  the  removal  of  already  existing  S3'mptoms. 

Internally  cinchona  preparations,  myrrh,  yeast  preparations  (zymin, 
Isevurinose  and  others)  may  be  tried. 

The  scorbutic  affection  of  the  gums  is  treated  locally  with  astrin- 
gents, painting  ^\'ith  weak  solutions  of  silver,  aluminium  acetate,  alum, 
chlorate  of  pota.sh,  potassium  permanganate  or  tincture  of  myrrh; 
older  cliildren  rinse  their  mouths  with  a  decoction  of  oak  bark  or 
cinchona.  The  skin  ulcers  require  antiseptic  bandages  (potassium 
permanganate),  avoiding  surgical  interference. 

Haemoglobinuria. — During  paroxj^sms,  rest  in  bed,  warmth,  avoid- 
ance of  lowering  body  temperature,  bland  diet,  plenty  of  milk  and 
water,  warm  baths.  In  the  intervals,  strengthening  of  the  body,  pro- 
tection against  colds  and  overexertion.  Should  there  be  a  recognized 
cause,  hereditary  syphilis  or  malaria,  then  the  medication  should  be  di- 
rected against  this  by  antisyphihtic  or  quinine  treatment,  respectively. 


INFANTILE  SCURVY 

BY 

Professou  W.  von  STARCK,  of  Kiel 

translated  by 
Dr.  CHARLES  K.  WINNE,  Jr.,  Albany,  N.  Y. 


(Synonyms. — Barlow's  Disease,  MoUer-Barlowsche  Krankheit.     Skorbut  der 
kleinen  kinder.     Infantile  Scurvy.     Scorbut  infantile.) 

Definition. — By  the  term  infantile  scurvy  is  understood  a  scorbutic 
affection  occurring  in  the  early  years  of  life,  and  characterized  cMefly  by 
marked  anajmia  of  the  skin  and  mucous  membranes,  bleeding  gums, 
pain  upon  movement  and  the  occurrence  of  swellings  upon  the  long 
bones  of  the  extremities  and  upon  the  ribs.  The  anatomical  basis  of  this 
disease  is  a  specific  affection  of  the  bone  marrow  associated  with  an£emia 
and  the  hsemorrhagic  diathesis.  In  the  majority  of  cases  the  disease 
appears  in  association  with  a  pre-existing  rachitis  of  .slight  or  severe 
grade,  but  it  may  occur  entirely  independently. 

History.— Mollcr  (1859  and  1862)  first  described  it  under  the  name 
"acute  rickets,"  as  he  believed  the  specific  symptom-complex  was  an 
indication  of  an  acute  exacerbation  of  rachitis,  though  Forster  was  in- 
cHned  to  a.ssign  to  it  an  independent  position.  Ingerslev  (1871)  and 
Jalland  called  it  scurvy,  though  some  EngUsh  authorities,  especially 
Cheadle,  laid  great  stress  upon  its  association  with  racliitis.  Barlow 
(1883)  first  brought  to  bear  upon  the  subject  numerous  pathological  as 
well  as  clinical  observations;  he  regarded  the  affection  as  scorbutic  and 
strongly  emphasized  the  importance  of  dietetic  therapy.  When  once 
the  attention  of  physicians  was  turned  to  the  question,  reports  appeared 
from  many  countries:  from  North  America,  Holland,  Denmark,  North 
Germany,  later  South  Germany,  France,  Belgium,  Sweden,  Austria, 
Switzerland,  Italy,  Finland,  etc.  The  American  and  French  physicians 
called  the  disease  scurvy,  those  from  other  countries  generally  Barlow's 
or  Moller-Barlow's  disease.  Heubner  wished  to  avoid  the  designation 
scurvy,  as  he  regarded  the  conception  of  scurvy  as  poorly  defined  and 
because  it  does  not  usually  occur  where  infantile  scurvy  is  frequently 
observed,  and  furthermore,  the  symptom-complex  of  infantile  scurvy 
differs  decidedly  from  that  of  the  adult  type  of  scurvy.  In  addition  to 
Barlow,  we  are  particularly  indebted  to  Naegeli,  Jacobsthal,  Schoedel- 
Nauwerk,   Schmorl    and    Frankel   for    the   demonstration    of   the    finer 

histological  changes  in  this  disease. 
186 


PLATE  8. 


II 


\i^ 


% 


:i  \ 


^:fc>^    ^ 


I.  Lower  leg,  9-months  infant,     a.  Subperiosteal  hspmorrhage  over  tibia  ;  6.  smaller  hcemorrhage  over  lower 
end  of  Ebula. 

II.  Femur,  same  child,    a.  subperiosteal  hsEmorrhage  ;  b,  juncture  of  shaft  and  epiphysis  ;  c.  hsemorrhage  in 
marrow. 

III.  Femur,  fracture  of  upper    end    of    the   diaphysis,   separation  from  epiphysis,     a,  periosteal  new  bone 
tissue. 

IV.  Section  of  broken  rib  in  process  of  healinp.    a,  subperiosteal  haemorrhage  ;  b,  periosteal  callus. 


INFANTILE    SCURVY  1S7 

Occurrence. — -Infantile  scurvy  is  distinctly  an  affection  of  artifi- 
ciall)'  fed  children  anil  though  it  has  greatl}'  increased  in  frequency  in 
the  last  twenty  to  thirty  years,  it  is  yet  rather  rare. 

Of  one  hundred  cases,  the  ages  at  the  beginning  of  treatment  were 
as  follows" 

1  case 4          inontlis 

1  "      5 

10  "      6 

10  •■      7 

20  •■      8 

17  "      9 

13  '■      10 

11  •'      11 

7     "      12 

7     "      13-18 

3     "      19-2-1 

Isolated  cases  have  been  noted  throughout  the  third  antl  fourth 
years;  the  oldest  case,  six  and  one  half  years,  was  autopsied  by  Friin- 
kel.  Boys  seem  to  be  affected  somewhat  more  frequently  than  girls. 
The  influence  of  season  is  uncertain.  The  occurrence  of  cases  in  Eng- 
land, Holland  and  Northern  Germany  speaks  for  a  geographical  and 
climatological  influence  though  cases  occur  in  all  countries.  Favorable 
social  conditions  predispose  to  the  occurrence  of  cases. 

Clinical  Picture. — The  symptoms  develop  gradually  and  at  first  are 
not  characteristic.     The  following  is  a  typical  chnical  history: 

A  child  in  good  suiToundings;  sunny  dwelUng,  garden,  careful  atten- 
tion; nourishment,  artificial  ^\^th  Gartner's  "Fat  milk."  Child  thrived 
until  the  ninth  month,  then  had  frequent  slight  digestive  disturbances; 
then  was  less  active  than  formerly,  and  dull.  There  was  an  increasing 
pallor  of  skin  and  mucous  membranes,  movements  of  the  body  were 
avoided;  the  child  cried  very  frequently  when  handled.  Legs  were 
held  as  if  paralysed.  About  the  upper  incisors  the  gums  were  much 
swollen  and  were  of  a  bluish  red  color  and  bled  easily. 

The  attending  physician  made  a  diagnosis  of  rachitis  and  ordered 
codliver  oil  and  phosphorus,  and  salt  baths.  The  cliild's  condition  gi-ew 
worse  under  tliis  treatment  and  it  was  therefore  brought  to  the  hosiMtal. 

Condition  on  admission,  November  11,  '03:  a  verj-  ana>mic  but 
moderately  well  nourished  girl  of  eleven  months  hes  immovable  on  the 
bed  and  cries  as  one  approaches  it.  No  signs  of  rachitis.  In  the  region 
of  the  upper  and  lower  middle  incisors  marked  htemorrhagic  sweUing  of 
the  gums;  at  the  lower  end  of  the  left  humerus  there  is  a  diffuse  painful 
sweUing  and  similar  ones  are  present  over  the  lower  third  of  the  right 
femur  and  the  lower  half  of  the  til)ia.  No  special  changes  in  heart  or 
lungs.     Temperature  38.5°  C.   (101.3°  F.). 

Blood  examination  shows:  hirmoglobin  50  per  cent.  (Gowers); 
shght  poikilocytosis,  marked  lymphocytosis,  no  abnormal  forms. 


188  THE    DISEASES   OF   CHILDREN 

Diagnosis. — Infantile  sicurvy. 

Dietetic  treatment  with  raw  cow's  niillc,  meat  juice  and  fruit  juice. 

Course. — After  fom'  days  there  was  a  decided  improvement  of  all 
symptoms,  the  child's  whole  condition  changed;  after  fourteen  da3's 
more  it  was  almost  well  and  was  taken  home.     Uneventful  recovery. 

Symptoms. — The  majority  of  the  symptoms  were  present  in  the 
above  case. 

1.  Anoemia. — Cliildren  formerly  l^right  and  rosy  become  gi-adually 
anaemic  and  finally  waxy-white.  The  examination  of  the  blood  shows 
a  fall  in  the  hjemoglobin  content  to  as  low  as  40  per  cent.,  a  marked  de- 
cline in  the  number  of  erythrocytes,  slight  poikilocytosis,  and  leucocy- 
tosis  with  a  decided  increase  in  the  mononuclear  at  the  expense  of  the 
polymorphonuclear  forms  (Ritter);  thus  relative!}^  insignificant  blood 
changes  with  absence  of  abnormal  forms.  The  \'iew  advanced  by  Sen- 
ator that  the  ansemia  is  the  result  of  a  primary  disease  of  the  bone  mar- 
row is  not  justified  by  the  pathological  changes  found  in  the  marrow 
and  the  blood. 

2.  Pain  on  Movement. — At  first  the  children  cry  very  often  with 
the  ordinary  handhng,  then  move  less  than  formerly  and  finally  every 
movement  or  even  a  touch  is  painful.  Movements  of  the  legs  are  at 
first  the  most  painful,  and  upon  careful  investigation  one  finds  especial 
tenderness  at  the  ends  of  the  diaphyses;  finally  the  legs  lie  immovable, 
as  in  sypliilitic  pseudoparalysis.  The  thorax  also  is  very  frequently 
tender,  the  arms  less  often  so.  Tliis  tenderness  of  the  bones  may  be 
absent  notwithstanding  other  well-marked  symptoms  of  infantile  scurvy. 
The  tenderness  which  often  extends  over  the  whole  body  is  dependent 
upon  pathological  changes  in  the  bones  and  less  upon  a  general  hyper- 
sesthesia. 

3.  Enlargement  of  the  Bones. — Swollen  areas  appear  upon  one  or 
more  bones,  most  frequently  at  the  lower  end  of  the  femur,  so  that 
these  bones  seem  locally  enlarged,  and  over  them  the  skin  becomes  tense 
and  glistening  and  the  swollen  part  feels  doughy  to  the  touch.  The  swell- 
ing seldom  reaches  above  the  lower  third  or  at  most  the  middle  of  the 
femur.  Frequently  both  thighs  are  involved.  No  less  frequently  the 
osseo-cartilaginous  junction  of  the  ribs  is  enlarged  so  that  the  picture 
of  a  racliitic  rosarj-  appears,  and  confusion  with  rickets  may  arise.  In 
severe  cases  of  rib  involvement  a  separation  of  the  cartilaginous  from 
the  bony  portions  of  these  bones  occurs,  so  that  the  sternum  and  adja- 
cent costal  cartilages  sink  bodily  backward.  This  phenomenon  is  almost 
specific  for  the  disease. 

Barlow  says  concerning  this:  "The  sternum  with  adjacent  costal 
cartilages  and  a  small  portion  of  the  contiguous  ribs  appear  as  though 
they  had  been  fractiu-ed  by  a  blow  from  the  front  and  had  been  forced 
backward." 


INFANTILE    SCURVY  189 

The  legs  swell  similarly  to  the  thighs,  and  in  association  with  the 
enlargement  at  the  upper  end  of  the  tibia  there  is  often  found  a  swelhng 
of  the  entire  lower  leg.  These  painful  swellings  may  appear  on  the  hu- 
merus as  well  as  on  the  bones  of  the  forearm,  the  scapula,  the  jaws  or 
any  bone  of  the  body.  After  they  remain  for  a  time  the  skin  over  them 
assumes  a  bluish  or  bluish  red  discoloration.  Not  infrequently  \\ith  or 
\vithout  these  enlargements,  evidences  of  interruption  of  continuity, 
crepitation  and  displacement,  appear  at  the  ends  of  the  diaphyses  of 
the  affected  bones. 

4.  Hcemorrhagic  swelling  and  softening  of  the  gums  is  a  very  impor- 
tant and  frequent  symptom.  The  dark  bluish  or  purplish  spongy  gum 
closely  surrounds  and  overlaps  the  teeth  and  shows  a  tendency  to  bleed. 
There  is  however,  no  tendency  to  destruction  of  the  gums  as  in  ordinary 
scurvy.  This  hsemorrhagic  change  is  noted  only  about  the  teeth  which 
have  already  appeared  or  around  those  which  are  about  to  come  through; 
in  fact,  in  the  depths  of  the  tumefaction  one  often  sees  the  points  of 
teeth  which  first  show  themselves  as  the  swelling  subsides.  In  children 
^vithout  teeth  this  change  is  either  not  seen  at  all  or  only  just  before  the 
teeth  are  cut. 

5.  Hemorrhagic  swelling  of  the  Eyelids  and  Exophthalmus. — Sub- 
periosteal extravasations  of  blood  appear  also  upon  the  cranial  bones 
especially  upon  those  of  the  orbit;  they  may  pre.ss  the  eyeballs  forward, 
and  through  infiltration  of  the  blood  into  the  loose  tissues  of  the  lids 
may  cause  the  latter  to  become  much  swollen  and  of  a  bluish  red  color. 
This  frequently  affects  both  eyes  and  produces  a  marked  disfigurement 
of  the  child. 

6.  Extravasations  of  blood  into  the  skin  and  mucous  membranes  are 
seen  as  further  evidences  of  the  ha?morrhagic  diathesis,  but  on  the  whole, 
they  are  less  frequent  and  are  but  slightly  characteristic.  Thus  there 
are  small  and  larger  haemorrhages  under  the  skin,  usually  in  locations 
subject  to  irritation  or  in  scars,  hsemorrhages  into  the  oral  mucosa  in 
addition  to  the  gingivitis,  into  the  conjunctiva,  the  nasal  mucosa,  and 
that  of  the  intestinal  tract  (bloody  stools"). 

7.  Hcematuria  occurs  in  ten  per  cent,  of  the  cases  (Heubner),  and 
is  sometimes  the  only  e\idence  of  the  hsemorrhagic  diathesis.  The 
urine  shows  a  large  amount  of  albumin,  numerous  erythrocytes  and 
granular  and  red  corpuscle  casts;  a  true  hipmorrhagic  nephritis  is  rare. 

8.  Fever. — The  temperature  in  about  fifty  per  cent,  of  the  cases  is 
slightly  elevated  without  definite  type,  and  in  general  seldom  rises 
above  39°  C.  (102.2°  F.).  The  duration  of  the  fever  is  very  variable; 
feverish  periods  alternate  sometimes  A\Tth  those  of  normal  temperature. 

No  characteristic  symptoms  referable  to  the  other  organs  occur. 
The  respiration  is  frequent  on  account  of  the  marked  anoeniia,  the  pulse 
is  accelerated,  the  heart  is  sometimes  dilated  and  anrpmic  murmurs  may 


1!)0  THE   DISEASES   OF   CHILDREN 

be  heard.  The  appetite  is  poor;  the  bowels  are  normal,  or  sluggish, 
though  there  may  be  diarrhoea  with  traces  of  blood-tinged  mucus,  espe- 
cially if  the  haemorrhage  into  the  intestinal  mucosa  stimulates  peristalsis. 
Bronchitis,  pneumonia  and  severe  intestinal  catarrh  are  frequently  met 
Avith  as  comphcations. 

The  course  is  decidedly  chronic.  Weeks  or  months  are  required  for 
the  full  development  of  the  clinical  picture,  and  then  the  condition 
fluctuates  backward  and  forward  until  death  supervenes,  apparently 
from  cardiac  weakness  often  aided  by  a  complicating  enteritis  or  pneu- 
monia. Or  a  correct  diagnosis  leads  to  proper  treatment  and  saves  the 
life  of  the  child.  Without  this  the  children  usually  die;  the  very  slight 
cases  may  recover  spontaneously.  Apparently  in  many  early  cases, 
perhaps  just  beginning,  a  simple  change  in  diet  undertaken  because 
the  children  were  pale  and  dull,  leads  to  recovery  without  infantile 
scurvy   being  suspected. 

Pathology. — Naegeli,  Schoedel-Nauwerk,  Schmorl  and  Friinkel 
agree  that  the  pathological  changes  in  infantile  scurvy  consist  chiefly 
in  a  characteristic  affection  of  the  bone  marrow  which  is  most  marked 
at  the  osseo-cartilaginous  border,  and  comprises  a  change  of  the  normal 
lymphoid  marrow,  which  is  rich  in  cells,  into  a  tissue  poor  in  cellular 
elements,  which  contains  but  few  blood  vessels  and  consists  of  a  homog- 
eneous ground  substance  containing  spindle  and  stellate  cells.  The 
transformation  of  the  marrow  with  the  associated  destruction  of  osteo- 
blasts, while  normal  bone  absorption  proceeds,  must  necessarily  result 
in  an  abnormal  thinness  and  insufficient  density  of  the  youngest  por- 
tions of  the  diaphysis,  at  the  margin  of  growth.  From  this  circumstance 
a  great  rarefication  of  the  bone  results  both  in  the  region  of  the  first 
lamella;  and  in  the  deeper  layers. 

Consequently  the  ends  of  the  shafts  of  the  diseased  bones  become 
brittle  on  account  of  the  thin  cortex,  the  scarcity  of  strong  trabecuhe 
and  the  persistence  of  much  calcified  ground  substance  which  has  not 
been  transformed  into  true  bone.  On  this  account  even  small  traumata, 
such  as  the  traction  of  the  muscles  at  their  attachments,  lead  to  par- 
tial or  complete  fractures  at  the  extremities  of  the  long  bones  and  to 
displacement  of  the  costal  cartilages  (see  Plate  8).  Breaks  very  rarely 
occur  at  a  great  distance  from  the  epiphyses,  as  in  the  shafts.  As  a 
result  of  the  fissures  and  fractures  at  the  epiphyseal  line,  the  epiphyses 
become  loosened  and  dislocated  but  no  true  epiphyseal  separation 
occurs.  Severe  displacement  of  the  fragments  is  prevented  by  the 
fact  that  the  periosteum  is  very  seldom  torn.  The  joints  always  remain 
unaffected. 

Subperiosteal  hannorrhages  of  varying  extent,  surrounding  the 
entire  shaft,  usually  accompany  the  breaks  in  the  bone  and  often  lead 
to   visible   and   palpable   swelling   of    the   limbs.      These   hasmorrhages, 


INFANTILE    SCURVY  I'Jl 

however,  may  be  absent  notwithstanding  severe  bone  lesions;  they  are 
dependent  upon  the  severity  of  the  htomorrhagic  diathesis  which  accom- 
panies the  bone  affection.  Tliis  leads  to  hiemorrhages,  not  only  about 
the  fractured  bones  but  also  on  other  bones,  especially  where  growth  is 
very  active,  e.g.,  the  jaws;  also  to  haemorrhages  in  the  bone  marrow, 
into  the  parenchyma  of  the  internal  organs,  and  into  the  intestinal 
mucosa  (diffuse  ecchymosis  of  the  mucosa  of  the  ileum,  Frankel).  In 
several  cases  which  had  had  hiematuria  Friinkcl  found  no  inflammatory 
changes  in  the  Iddneys  but  merely  haemorrhages  into  the  tissue. 

According  to  the  same  authority  radiographs  of  the  diseased  bones 
show  characteristic  features;  in  the  lower  portions  of  the  diaphyses,  in 
place  of  the  fine  meshwork  of  the  spongiosa  there  occurs  a  washed  out 
space  \vith  irregular  margins.  If  the  case  recovers  tliis  disappears  only 
after  some  months.  Breaks  in  continuity  and  subperiosteal  haemorrhages 
are  ea.sily  recognized.     (For  a  personal  observation  see  Plate  9). 

After  the  absorption  of  the  necrotic  material  at  the. point  of  frac- 
ture (the  "Triimmelfeld"  zone  of  Frankel)  the  regeneration  of  the  bone 
takes  place  through  the  appearance  of  small  masses  of  normal  lymphoid 
marrow  cells  in  the  pathologically  rarefied  marrow,  and  the  replacement 
or  removal  of  the  latter  by  their  gradual  growth.  After  that  the  forma- 
tion of  new  bone  proceeds  normally  and  strong  osseous  trabeculae  are 
formed.  If  marked  dislocation  occurs  after  a  fracture  a  deformity  may 
remain  in  the  neighborhood  of  the  joint. 

Relationship  to  Rachitis. — -Schoedel  and  Nauwerk  beUeve  that  ra- 
chitis plays  a  special  role  in  infantile  scurvy;  on  the  other  hand,  Naegeh, 
Schmorl,  Stooss  and  Frankel  consider  them  as  independent  affections, 
though  they  recogmze  their  frequent  association  which  may  be  explained 
by  the  children's  age  and  the  artificial  feeding.  Cases  of  infantile  scurvy 
of  the  severest  grade  exist  without  a  trace  of  rickets,  and  the  anatomical 
changes  in  the  two  conditions  are  essentially  different. 

The  question  whether  or  not  infantile  scurvy  is  to  be  con.sidered  as 
scurvy  cannot  be  decided  until  we  possess  satisfactory  reports  upon  the 
histology  of  the  bone  changes  in  the  latter  disease.  The  macroscopic 
lesions  seem  to  be  very  similar  (Netter,  Stooss).  At  all  events,  chni- 
cally,  infantile  scurvy  and  scurvy  are  closely  related  and  the  majority 
of  physicians  are  inchned  to  regard  the  two  diseases  as  practically  one. 
From  a  scientific  standpoint  the  decision  will  first  be  made  when  the 
above-named  condition  is  fulfilled. 

Etiology. — The  specific  cause  of  infantile  scurvyis  as  yet  unknown. 
Two  factors  play  the  principal  roles  in  its  causation:  (1)  the  kind  of  food 
the  cliild  has  had  and  (2)  a  special  indi\'idual  susceptibihty. 

Only  artificially  fed  children  are  affected,  and  the  unsuitable  diet, 
which,  considering  the  needs  of  the  child,  is  insufficient,  must  have  been 
maintained  for  several   months.     Whether   breast-fed   children  can  be 


192  THE    DISEASES   OF   CHILDREN 

affected  is  doubtful;  the  few  cases  of  this  kind  reported  in  the  hterature 
are  not  free  from  criticism.  As  severe  a  grade  of  malnutrition  can  occur 
with  mother's  milk  as  with  artificial  feeding  when  the  breast-milk  does 
not  supply  the  special  needs  of  the  suckling  (autointoxication,  Variot). 

The  loss  of  certain  fresh  properties  in  the  milk,  through  heating  it, 
is  one  of  the  most  important  causes  of  tliis  affection,  and  other  impor- 
tant factors  are  insufficient  feeding  and  monotony  in  diet. 

•Individual  pretUsposition  is  shown  by  the  fact  that  of  twins  who 
have  had  the  same  nourishment  one  may  thrive  splendidly  and  the  other 
become  affected.  Finkelstein  saw  an  infant  ill  with  the  disease  who, 
because  a  brother  had  formerly  suffered  with  the  same  complaint,  had 
received  only  milk  heated  for  a  short  time,  and  fresh  vegetables. 

Infantile  scurvy  occurs  with  all  forms  of  artificial  feeding  but  cer- 
tain methods  favor  its  appearance.  SteriUzed  and  prepared  milk  of 
various  sorts  come  first,  then  pasteurized  milk  and  simple  boiled  milk, 
then  milk  and  flour  mixtures  and  prepared  flour  alone,  and  finally 
oatmeal  gruel  and  rice  gruel.  With  the  use  of  raw  cow's  milk  the  disease 
is  rather  rare. 

The  manifold  attempts  to  give  to  cow's  milk  a  "human  character," 
the  undue  valuation  of  its  special  natural  properties  and  the  over 
valuation  of  the  modification  of  its  gross  composition  to  as  near  as 
possible  that  of  human  milk,  have  all  favored  the  increased  occurrence 
of  this  formerly  almost  unknown  disease.  The  more  frequent  occur- 
rence of  the  affection  in  the  families  of  the  rich  than  in  those  of  the  poor 
is  explained  by  the  fact  that  specially  prepared  milk  and  the  many 
proprietary  foods  are,  on  account  of  their  high  price,  more  accessible  to 
the  well-to-do  than  to  those  in  less  easy  circumstances.  Besides  this, 
an  undesirable  uniformity  of  food  is  not  infrequent  in  the  diet  lists  of 
well-to-do  families. 

One  should  be  prepared  to  meet  infantile  .scurvy  everywhere,  among 
poor  and  rich  alike.  Cheadle  noted  the  relative  immunity  of  the  chil- 
dren of  the  poor,  and  ascribed  this  fact  to  the  circumstance  that  early  in 
life  these  children  subsist  on  fresh  food  added  to  their  milk.  In  cases  of 
this  kind  continued  underfeeding  with  oatmeal  gruel,  rice  gruel,  etc., 
has  sometimes  taken  place,  but  in  general  the  caloric  value  of  the 
food  which  preceded  the  appearance  of  infantile  scurvy  has  been  more 
nearly  sufficient. 

Among  the  unavoidable  changes  which  take  place  in  milk  when  it 
is  heated,  and  which  have  been  considered  as  etiological  factors  in  in- 
fantile scurvy  are:  (1)  the  destruction  of  a  certain  amount  of  nucleon- 
phosphorus;  (2)  the  destruction  of  all  enzymes;  (3)  the  change  of  soluble 
calcium  compounds  into  insoluble  calcium  phosphate;  (4)  the  conversion 
of  a  certain  amount  of  the  amorphous  neutral  calcium  citrate  into  the 
less  soluble  crystalUne  form.    Netter  considers  citric  acid  as  the  specific 


INFANTILE  SCURVY  193 

antiscorbutic  constituent  of  cow's  millv,  but  as  the  latter  is  much  riclier 
in  citric  acid  than  is  mother's  milk,  a  deficit  cannot  easily  occur  even  with 
cooking. 

Johannessen,  in  conformity  with  the  recent  theory  that  marine 
scurvy  is  due  to  an  intoxication,  suggests  that  toxins  from  the  killed 
bacteria  in  the  milk  may  have  a  part  in  the  production  of  infantile 
scurvy.  Neumann  seeks  the  cause  in  a  chronic  poisoning:  "The  poison 
may  arise  exogenously  from  the  food  by  bacterial  action,  by  chemical 
means  or  by  the  action  of  heat,  or  it  may  arise  endogenously  during  diges- 
tion." In  the  conclusions  which  are  drawn  from  the  collective  studies 
of  the  question  by  the  American  Pediatric  Society  the  possibility  of  an 
autointoxication  is  suggested.  The  suppo.sition  that  infantile  .scurvy  is 
due  to  some  toxin  ari.sing  in  the  food  and  that  this  affects  only  certain 
susceptible  children  while  the  great  majority  thrive  on  the  same  nour- 
ishment would  most  easily  explain  the  whole  symptom-complex,  and  the 
prompt  action  of  dietetic  therapy,  the  result  of  a  simple  change  in  diet. 

Microscopic  exainination  of  the  blood  and  other  tissues,  and  special 

bacteriological  experiments  (Schmorl)  have  so  far  given  no  support  to 

.  the   theory   of  a   direct   bacterial   origin   of  the  disease,  nor  have  any 

results  been  derived  from  its  attempted  artificial  production  in  animals 

(Bartenstein). 

Diagnosis. — -If  one  carefully  considers  the  symptoms  which  have 
already  been  described,  tliis  disease  will  hardly  be  mistaken  for  any  other, 
but  it  is  of  great  importance  to  make  the  diagnosis  before  the  disease 
gains  much  headway.  If  in  a  bottle-fed  infant  a  progressively  severe 
anaemia  develops  with  a  coexistent  suspicion  of  htemorrhagic  swelling 
of  the  gums,  and  tenderness  at  the  epiphyseal  ends  of  the  long  bones 
one  should  tliink  of  Barlow's  disease — ^infantile  scurvy. 

Mistakes  frequently  occur  through  the  observation  of  marked  uni- 
lateral swellings  on  the  long  bones;  the  diagnosis  of  periostitis,  ostitis, 
osteomyelitis,  osteosarcoma,  etc.,  is  made,  even  operations  of  greater 
or  less  magnitude  are  undertaken  without  result,  until  the  death  of  the 
child  or  the  di.scovery  of  subperiosteal  hsemorrhages  puts  one  on  the 
right  track.  The  entire  clinical  picture  should  not  be  neglected,  the 
severe  ansemia,  and  its  gradual  development  should  be  sufficiently 
appreciated;  the  entire  child  should  be  examined. 

In  contrast  to  severe  aniemias  from  other  causes  with  a  tendency 
to  the  occurrence  of  hajmorrhages,  it  is  important  to  remember  that 
aside  from  a  considerable  reduction  in  the  percentage  of  htemoglobin 
the  blood  changes  in  infantile  scurvy  are  not  characteristic  (see  above). 

Infantile  .scurvy  is  readily  liidden  behind  an  associated  racliitis,  or 
may  be  mistaken  for  rachitis,  though  the  latter  does  not  exist  at  the 
time.  For  tlus  reason  the  progressive  an;rmia,  the  affection  of  the 
gums,  and  the  painful  swellings  on  the  long  bones  are  all  very  impor- 

11—13 


194  THE   DISEASES   OF   CHILDREN 

tant.  Swelling  and  sensitiveness  at  the  osteocartilaginous  border  of 
the  ribs  is  common  to  both  diseases;  an  angular  fracture  between  the 
prominent  bony  part  and  the  depressed  cartilage,  or  possibly  even  a 
depression  of  the  sternum  together  with  the  cartilaginous  portion  of  the 
ribs  speaks  for  infantile  scurvy.  In  congenital  syphilis  sweUings  similar 
to  those  of  infantile  scurvy  appear  on  the  long  bones,  and  the  condition 
of  pseudoparalysis  is  frequent  in  congenital  syphihs,  marked  ansemia 
also  occurs,  but  in  addition  there  are  the  other  usual  symptoms  of  syph- 
ihs. The  pecuHar  gingivitis  and  eventually  the  other  signs  of  the  ha?mor- 
rhagic  diathesis  are  very  valuable.  Radiographs  of  the  diseased  bones 
can  be  of  especial  service  in  difficult  cases. 

Incipient  and  abortive  examples  of  infantile  scurvy  make  them- 
selves evident  by  the  increasing  aniemia,  the  restlessness  and  the  hyper- 
sesthesia  of  the  children. 

Prognosis. — In  simple  cases  the  prognosis  is  favorable  if  the  diag- 
nosis is  made  and  dietetic  therapy  instituted;  but  if  a  complicating 
intestinal  catarrh  or  bronchopneumonia  exists,  or  if  the  cardiac  strength 
of  the  child  has  already  seriously  suffered,  the  prognosis  is  doubtful  in 
spite  of  proper  feeding. 

The  mortality  is  still  high,  as  the  diagnosis  is  often  not  made,  though 
it  is  to  be  hoped  that  with  increasing  knowledge  of  the  characteristic 
symptoms,  the  abihty  to  make  the  diagnosis  will  be  improved,  and 
with  this  the  prognosis. 

Prophylaxis. — If  a  marked  ana-mia  develops  with  artificial  feeding 
it  is  advisable,  in  order  to  rectify  the  diet,  to  give  fresh  food  occasionally. 
Sterilized  or  prepared  milk  should  not  be  given  over  a  long  period  of 
time  without  the  addition  of  fresh  fruit  or  vegetables;  and  in  general 
fresh  briefly  boiled  milk  should  be  used.  Uniformity  of  diet  for  many 
months  should  be  avoided  and  malnutrition  corrected  as  soon  as  possible. 

Treatment. — The  treatment  of  this  disease  is  generally  as  easy  as 
it  is  satisfactory.  A  proper  change  in  diet,  without  the  help  of  any 
medication,  leads  in  a  short  time,  even  quicker  than  in  ordinary  scurvy, 
to  a  complete  revolution  in  the  ol^jective  and  subjective  condition  of 
the  patient.  Severe  conditions  and  menacing  appearances  diminish  in 
an  almost  magical  manner.  In  place  of  the  food  which  has  heretofore 
bfeen  given,  the  child  should  receive  fresh,  at  most  briefly  heated,  or 
still  better  raw,  cow's  milk,  if  such  is  to  be  had  from  a  rehable  source. 
Besides  tliis  two  to  four  teaspoonfuls  per  day  of  raw  meat  juice  should 
be  givien,  and  the  same  amount  of  fresh  fruit  juice  (obtained  according 
to  the  season  of  the  year  from  oranges,  grapes,  lemons,  cherries,  cur- 
rants, blackberries,  apples,  pears,  apricots,  huckleberries,  etc.)  sweet- 
ened with  sugar. 

To  children  in  the  second  or  third  years  one  can  give  in  addition 
to  the  fresh  milk,  potato,  vegetable  soup  (carrots,  cabbage,  cauliflower, 


9^ 


I.  Upper  extremities  and  pelvis  of  a  12-montbs  infant  with  typical  infantile  scun-y. 
II.  Left  arm  of  the  same  ease. 


INFANTILE  SCURVY  195 

spinach),  stewed  fruit,  green  salads,  and  finally  chopped  meat.  The  food 
is  usually  taken  eagerly.  After  one  or  two  weeks  the  symptoms  will 
for  the  most  part  have  disappeared.  If  gastric  or  intestinal  catarrh 
exists  one  may  give  briefly-boiled  milk  with  oatmeal  gruel,  currant, 
blackberry  or  elderberry  juice,  chicken  jelly,  or  better  still,  breast-milk, 
even  to  children  over  one  year  of  age. 

The  tenderness  of  the  limbs,  the  fractures,  etc.,  demand  rest,  avoid- 
ance of  all  unnecessary  handhng,  and  local  applications.  The  complete 
repair  of  the  bone  lesions  may  be  delayed  for  several  months. 


RACHITIS 

BY 

Prof.  W.  STOELTZNER,  of  Halle 

translated  by 
Dr.  CHARLES  K.  WINNE,  Jr.,  Albany,  N.  Y. 


Occurrence. — Rachitis  is  an  extraordinarily  wide-spread  disease.  It 
occurs  most  frequently  in  the  civilized  portions  of  the  north  temperate 
zone,  where  in  the  poorer  quarters  of  great  cities  it  affects  90  or  more 
per  cent,  of  all  children.  It  is  comparatively  infrequent  in  the  tropics 
on  the  one  hand  and  in  the  far  north  on  the  other  and  also  at  liigh 
altitudes.  It  is  very  rare  among  the  cliildren  of  the  yellow  races,  but 
although  negro  children  in  the  tropics  remain  practically  exempt  those 
in  regions  in  which  it  is  endemic  seldom  escape. 

Symptoms. — The  most  characteristic  symptoms  of  rachitis  are  those 
of  the  osseous  system;  in  addition  to  these  certain  general  manifesta- 
tions wliich  are  apt  to  be  most  evident  occur  in  the  majority  of  cases 
at  the  beginning  of  the  disease  at  a  time  when  the  bony  changes  are  not 
yet  clinically  evident. 

(a)  Constitutional  Symptoms. — These  are  quite  ambiguous.  The 
cliildren  become  restless,  ill-tempered  and  do  not  sleep  as  well  as  usual. 
If  they  lie  on  their  back,  especially  in  sleep,  they  roll  their  heads  from 
side  to  side  or  burrow  them  into  the  pillow,  though  if  they  lie  on  the  side 
they  usually  remain  quiet.  Children  over  six  months  of  age  with  this 
disease  learn  to  seek  the  latter  position  of  their  own  accord,  not  infre- 
quently gradually  lying  even  partially  or  wholh^  on  their  faces.  When 
such  a  child  is  carried  it  is  unable  to  hold  its  head  erect  for  more  than  a 
short  period  of  time,  and  usually  supports  its  cheek  or  face  against  the 
face  or  shoulder  of  the  nurse.  Oftentimes  the  friction  of  the  head  on 
the  pillow  leads  to  baldness  of  the  occiput. 

Another  important  symptom  is  sweating.  This  affects  the  head, 
principally,  where  it  is  often  so  marked  that  in  the  morning  the  child's 
pillow  is  soaked  through.  The  sweat  is  of  a  clammy  nature,  with  an 
acid  odor  and  reaction.  Oftentimes  sudamina  or  eczema  follow  in  the 
wake  of  the  excessive  sweating.  Coincidentally  the  vasomotor  excita- 
bility of  the  sldn  undergoes  an  abnormal  increase,  so  that  red  spots 
appear  in  a  short  time  over  any  area  where  friction  is  exerted.  Very 
frequently  the  urine  assumes  an  offensive  penetrating  odor.  In  chil- 
dren old  enough  to  run  about  the  absence  of  desire  to  do  so  is  often  a 
very  early  symptom  of  the  disease. 

If  these  initial  symptoms  are  very  marked  the  children  lose  their 

196 


RACHITIS 


197 


Fig.  31. 


fresh  appearance  and  become  more  or  less  pale  and  flabby.  In  many 
cases  the  constitutional  symptoms  are  so  sUght  that  the  disease  begins 
apparently  mth  those  referable  to  the  bones,  though  the  former  are 
usually  manifest  at  least  two  or  three  weeks  before  the  undoubted  bony 
changes  are  e\'ident. 

(6)  Osseous  System. — The  bone  changes  due  to  rachitis  may  be 
comprised  in  the  terms  "softening"  and  "deformity."  The  softening 
affects  principally  the  flat 
bones  and  the  diaphyses  of 
the  long  bones,  but  the  de- 
formity may  affect  any  bony 
part.  They  are  constant  even 
in  cases  in  which  gross  defor- 
mity never  appears.  As  the 
result  of  the  disturbance  of 
the  bony  growth  the  epiphyses 
of  the  long  bones  enlarge,  es- 
pecially at  the  points  of  union 
between  the  bony  and  carti- 
laginous fibs  where  they  can 
become  so  marked  that  the^' 
appear  as  large  nodules  lying 
under  the  skin.  The  skin  not 
infrequently  forms  a  deep  fold 
in  the  plane  of  the  articulation, 
especialh'  at  the  wrists. 

In  the  majority  of  the 
more  chronic  cases  a  deform- 
ity of  the  diaphyses  is  gradu- 
ally superimposed  upon  the 
enlargement  of  the  epiphyses. 
Such  deformities  frequently 
take  the  form  of  marked  exag- 
geration of  the  normal  bony 
curves,  but  the  essential  con- 
dition underlying  these  im- 
portant changes  of  form  is 
always  some  trauma  which  bends,  or  much  more  rarely  fractures,  the 
abnormally  soft  bones.  Marked  displacement  of  the  fragments  is  usually 
absent,  even  with  complete  fractures,  since  the  continuity  of  the  tliick- 
ened  periosteum  almost  always  remains  intact.  The  consolidation  of 
the  callus  is  much  delayed  by  rachitis.  The  force  wliich  is  necessarj' 
to  bend  or  break  a  rachitic  bone  is  in  inverse  ratio  to  the  extent  of  the 
bony  softening.    In  the  worst  cases  therefore  very  careful  protection  of 


Rachitis,    T}iree-ypar-oM  girl  with  rachitic  ro.sarv  and 
ga&eou^  distent iun  of  abdomen. 


198  THE    DISEASES   OF   CHILDREN 

the  cliild  is  necessary.  Often  in  a  very  short  time  the  bones  become 
markedly  softened,  even  under  the  eyes  of  the  observer. 

In  cases  of  less  marked  bony  softening,  partial  fractm-es  can  take 
place  during  powerful  muscular  contraction,  as  for  example,  in  the 
forearm  following  tetanic  or  eclamptic  convulsions. 

In  almost  all  cases  in  wliich  the  disease  has  lasted  for  a  number  of 
months  the  body  length  of  rachitic  children  is  less  than  the  normal 
average  for  their  age.  In  such  cases  the  shortening  of  the  bones  is  caused 
not  only  by  the  bowing  but,  at  least  in  the  more  severe  cases,  by  a  true 
injury  to  the  longitudinal  growth  especially  in  the  bones  of  the  lower 
extremities.  In  many  cases  there  develops  toward  the  end  or  after  the 
cessation  of  the  active  stage,  an  almost  complete  absence  of  growth 
wliich  leads  to  dwarfing. 

An  impairment  of  the  motor  functions  usually  coexists  with  this 
deformity  of  the  skeleton.  Only  a  minority  of  rachitic  children  learn  to 
stand  or  walk  at  a  period  approaching  the  normal;  -u-ith  most  of  them 
the  time  is  postponed  for  months  or  even  years.  Cliildren  that  are  walk- 
ing at  the  onset  of  the  disease  quickly  lose  this  function.  In  addition  to 
the  fact  that  racliitic  cliildren  do  not  learn  to  stand  or  walk  at  the  proper 
time,  the  majority  of  them  use  their  legs  very  httle  or  indeed  not  at  all, 
and  if  one  attempts  to  place  them  upon  their  feet  their  knees  strike  to- 
gether. If  the  legs  are  not  used  at  all  they  remain  as  a  rule  free  from 
deformity,  and  in  such  cases  the  epiphyseal  enlargement  frequently 
does  not  occur.  The  upper  extremities  are  remarkably  spared  in  many 
children,  but  in  very  severe  cases  they  too  are  very  Httle  used. 

This  di.sinchnation  to  move  is  associated  with  a  striking  laxity  of 
the  voluntary  muscles.  The  physiological  tone  is  wanting  in  these  cases, 
and  as  a  result  the  hmbs  of  such  children  can  be  easily  placed  in  posi- 
tions that  are  otherwise  impossible  except  in  the  case  of  so-called 
"snake  men"  or  sometimes  in  individuals  with  wide-spread  atrophic 
paralysis.  This  muscular  hypotonicity  is  greatly  misinterpreted,  and 
racliitic  children  are  described  as  having  an  abnormal  laxity  of  the 
periarticular  hgaments.  Hagenbach-Burkhardt  refer  to  this  abnor- 
mal hypotonicity  as  a  specific  (racliitic)  weakness  of  the  muscles  but  I 
am  far  more  inclined  to  regard  it  as  an  interception  of  the  innervation. 
In  many  cases  the  rachitic  bones  are  distinctly  sensitive  to  pressure, 
but  even  where  this  is  not  so,  the  muscular  contractions  can  cause  very 
severe  bone  pains,  because  at  such  time  the  tendons  necessarily  exert 
traction  on  the  softened  tliickened  periosteum,  upon  which  they  are 
inserted.  It  is  very  easy,  therefore,  to  suppose  that  children  learn  to 
inhibit  any  nervous  stimulus  which  might  bring  pain  in  its  wake.  In  the 
later  stages  of  the  disease  the  deformities  may  mechanically  render 
motion  difficult. 

Only  in  rare  cases  does  faulty  nutrition  cause  a  backwardness  in 


RACHITIS 


199 


Fig.  32. 


learning  to  stand  or  walk.  Often  when  rachitic  children  do  finally  learn 
to  walk  they  have,  in  severe  cases  after  a  year  or  so,  a  waddling  gait  and 
become  easily  tired  on  account  of  the  deformity  of  the  pelvic  girdle  and 
of  the  lower  extremities. 

Rachitis  very   frequently  load.-<   to   the   following  striking  clinical 
changes  on  the  part  of  individual  portions  of  the  skeleton. 

A  disproportion  between 
the  face  and  head  often  exists 
due  to  the  fact  that  the 
growth  of  the  facial  bones  is 
slower  than  that  of  the  bones 
of  the  skull.  This  dispropor- 
tion is  especially  striking  in 
the  case  of  older  individuals. 
In  rachitis  the  transverse 
diameter  of  the  upper  jaw  is 
decreased  but  is  definitely 
increased  in  the  sagittal  plane 
so  that  it  projects  forward 
in  the  mid-fine  hke  a  beak. 
In  contrast  to  this  the  under 
jaw  is  shortened  in  the  sagit- 
tal plane:  the  physiological 
curve  in  the  region  of  the  in- 
cisor teeth  is  flattened,  and 
the  normal  curve  in  the  re- 
gion of  the  canine  teeth  be- 
comes angular.  The  lateral 
portions  of  the  jaw  converge, 
while  its  thicker  lower  edge 
turns  upward  and  the  alveo- 
lar margin  bends  inward. 
The  incisor  teeth  are  often 
bent  sharply  inward  so  that 
when  the  mouth  is  closed 
there  remains  a  space  of  sev- 
veral  miUimetres  between  the  fingual  surface  of  the  upper  and  the  labial 
surface  of  the  lower  ones.  The  inner  surface  of  the  upper  molars  often 
rests  against  the  outer  surface  of  the  lower  molars.  As  a  consequence 
of  the  lack  of  development  of  the  alveolar  process  there  not  infrequently 
results  a  lack  of  sufficient  space  for  the  teeth. 

In  almost  all  cases  dentition  is  prolonged  by  racliitis ;  oftentimes 
children  begin  their  second  year  with  no  teeth,  although  the  termina- 
tion of  the  first  dentition  in  such  children  usuallv  occurs  in  the  third 


Racliitic  scoliosis.     Tliree-\far-oM  gill. 


200  THE   DISEASES   OF   CHILDREN 

3'ear.  A  characteristic  phenomenon  is  the  cutting  of  many  teeth  in  close 
succession  after  a  considerable  period  of  time.  As  a  rule  racliitic  teeth 
are  less  resistant  than  normal  ones,  and  the  enamel  often  shows  horizon- 
tal and  longitudinal  striations  or  roundish  depressions.  Frequentlj'  the 
teeth  are  discolored;  they  may  be  yellowish,  brown,  black  or  greenish 
gray.  They  are  inclined  to  become  carious  and  to  fall  out,  or  they  grad- 
ually crumble  down  to  the  alveolar  edge.  Sometimes  only  the  upper 
teeth  behave  in  this  manner  while  those  in  the  lower  jaw  remain  intact. 

In  the  cranium,  long  .standing  rachitis  leads  to  a  thickening  of  the 
frontal  and  parietal  tuberosities  which,  with  marked  prominence  of  the 
tubera  frontaha  and  a  simultaneous  flattening  of  the  occiput,  gives  a 
cubical  or  dice-hke  shape  to  the  skull.  These  are  cases  of  so-called 
"caput  quadratum"  or  square  head. 

In  rachitic  children  the  large  fontanelle  often  reaches  an  abnormal 
size,  and  is  often  closed  only  at  the  end  of  the  second  year  or  even  later. 
The  small  fontanelle  and  the  two  posterior  lateral  fontanelles  remain 
open  in  many  cases  an  abnormally  long  time,  as  do  the  longitudinal, 
the  coronal  and  the  lambdoidal  sutures.  The  bony  edges  of  the  large 
fontanelle  are  often  so  soft,  either  partially  or  throughout  their  whole 
extent,  that  they  can  be  easily  depressed.  Often  other  places  in  the 
skull  are  soft  and  compres.sible.  This  so-called  " craniotabes"  occurs 
principally  in  the  region  of  the  lambdoidal  suture  and  the  small  fonta- 
nelle; it  may  however  extend  far  into  the  occipital  bone  or  into  the 
posterior  part  of  the  parietal  bones.  The  soft  areas  are  usually  about 
the  size  of  a  dime  but  they  not  infrequently  unite  to  form  larger  areas, 
and  in  the  severest  cases  the  softening  affects  almost  the  entire  occiput. 
The  latter  is  flattened  in  all  severe  cases.  The  temporal  veins  are  fre- 
quently markedly  dilated  in  severe  cranial  racliitis. 

The  racliitic  spinal  curvature  as  a  rule  does  not  form  an  angular 
projection  but  rather  a  more  or  less  gentle  bend.  Most  frequently  one 
finds  a  kyphosis  which,  in  typical  cases,  involves  the  lower  dorsal  and 
lumbar  vertebra.  Next  in  frequency  is  the  dextro-convex-dorsal  scoU- 
osis;  as  a  rule  a  second  curve  is  present  compensatory  to  the  first. 

In  the  tJwrax  the  most  constant  symptom  of  rachitis  is  the  occur- 
rence of  a  row  of  bead-like  enlargements  which  run  from  above  anteri- 
orly to  below  laterally  at  the  junction  of  the  bony  and  cartilaginous 
portions  of  the  ribs,  the  so-called  rachitic  "rosary."  These  enlargements 
are  more  pronounced  on  the  lower  than  on  the  upper  ribs;  that  upon 
the  11th  rib  occurs  normally  in  about  the  mid-axillary  line.  On  closer 
examination  each  single  nodule  consists  usually  of  two  swellings  sepa- 
rated by  a  furrow,  one  of  which  l^elongs  to  the  osseous  and  the  other  to 
the  cartilaginous  part  of  the  rib.  In  emaciated  children  the  rosary  is 
plainly  visible  but  in  fat  or  even  well  nourished  indi\iduals  it  can  be 
demonstrated  only  by  palpation. 


RACHITIS 


201 


202 


THE   DISEASES   OF   CHILDREN 


In  the  majority  of  severely  affected  children  a  still  further  mal- 
formation of  the  thorax  is  associated  wath  the  rosary.  The  most  impor- 
tant visible  e\'idence  of  this  is  the  flattening  of  the  lateral  chest  wall. 
This  flattening  is  most  marked  as  a  rule  between  the  mammary  and 
postaxillary  lines  and  between  the  4th  and  7th  ribs.  In  severe  cases, 
in  place  of  the  normal  bowing  of  the  lateral  chest  wall  with  the  convex- 
ity outward,  a  more  or  less  deep  hollow  is  present,  so  that  here  the  ribs 
describe  a  curve  wth  a  concavity  outward.     In  contrast  to  the  lateral 


Fig.  34. 


Deformity  of  the  thorax,  pelvis,  and  extremities. 


wall  of  the  chest  the  sternum  in  these  cases  usually  projects  forward, 
and  if  a  kypho,sis  exists  at  the  same  time  the  sternum  curves  convexly 
forward  from  above  downward.  In  rare  cases  the  deformity  does  not 
assume  the  classical  form  of  the  racliitic  "chicken  breast,"  but  with  the 
typical  flattening  of  the  chest  wall  the  sternum  hes  somewhat  depressed 
between  the  very  prominent  costal  cartilages.  The  posterior  surface  of 
the  racliitic  thorax  is  abnormally  flattened  and  the  borders  between  the 
posterior  and  lateral  walls  are  usually  prominent.  In  contrast  to  the 
flattening  of   the  central   portions  of   the   chest   wall,  the   lowest  part 


RACHITIS 


203 


projects  outward,  the  sharp  transition  from  one  to  the  other  forming  the 
so-called  "Harrison's  grooves"  which  run  around  tlie  chest  at  the  level 
of  the  diaphragm. 

As  a  whole  the  chest  appears  strikingly  small  in  its  upper  two- 
tliirds,  especially  in  comparison  with  the  head  and  belly.  The  deformity 
of  the  thorax  in  racliitis  is  caused  on  the  one  hand  by  the  abnormal 
weakness  of  the  chest  wall  which  yields  in  time  to  the  diaphragmatic 
contractions  at  each  inspiration  and  to  the  elastic  pull  by  the  collapsing 
lungs  at  expiration,  and  on  tlie  other  to  the  continual  pressure  exerted 
by  the  overlying  upper  arms.  The  flattening  of  the  posterior  chest  wall 
is  the  result  of  the  continuous  dorsal  decubitus.  The  bulging  of  the 
costal  margin  is  caused  Iiy  tlie  rachitic  enlargement  of  the  liver. 


Fir..   3.1. 


Bead-like  fingers. 


In  this  disease  the  normal  curves  of  the  clavicle  are  pathologically 
increased;  frequently  the  clavicle  is  the  seat  of  angular  partial  fractures 
which  affect  chiefly  the  middle  anteriorly  convex  curve  of  the  bone. 
The  humerus  is  visibly  curved  only  in  severe  cases;  the  convexity  of 
the  curve  is  as  a  rule  outward  and  somewhat  backward.  The  enlarge- 
ment of  the  proximal  epiphyses  causes  notliing  special  to  be  noted  in 
the  living  child,  but  that  of  the  distal  ones  is  quite  often  easily  palpable 
and  in  emaciated  children  is  visible.  The  humerus  frequently  shows 
partial  or  complete  fractures,  usually  about  in  the  middle  of  its  diaphy- 
sis,  and  later,  a  marked  formation  of  callus.  The  length  of  the  upper 
arms  may  be  shortened  as  much  as  one-half,  partly  through  inliibition 
of   the   longitudinal   growth,    partly   through   bending   and   angulation. 


204 


THE   DISEASES   OF   CHILDREN 


In  the  forearm,  the  enlargement  of  the  epiphyses,  especially  the  distal 
ones,  is  the  most  constant  symptom.  The  diaphyses  frequently  show  a 
convex  curve,  the  most  prominent  part  of  which  usually  hes  in  the 
distal  half  of  the  forearm,  and  in  addition  a  spiral  bend  of  the  radius 
about  the  ulna  is  often  found  which  causes  a  more  or  less  marked 
permanent  pronation  of  the  hand.  The  phalanges  are  frequently 
pjo  3g  thickened   and  spindle-shaped, 

causing  the  fingers  to  assume  the 
appearance  of  a  string  of  beads. 
Of  the  rachitic  deformities  of 
the  infantile  pelvis  the  thicken- 
ing of  the  iliac  crests  has  the 
greatest  cUnical  interest.  In  the 
femur  the  distal  epiphysis  is  often 
markedly  thickened,  and  the  bone 
is  bent  with  the  convexity  as  a 
rule  forward  or  outward.  Par- 
tial fractures  are  not  infrequent 
liere  and  usually  occur  in  the  mid- 
dle of  the  diaphysis.  In  the  bones 
of  the  lower  leg  the  distal  epiphy- 
ses as  a  rule  enlarge  more  mark- 
edly than  the  proximal  ones.  Very 
(jften  partial  fractures  are  found 
here  also,  usually  situated  in  the 
lower  third  with  their  convexity 
towards  the  front  and  outer  side, 
liesides  these  fractures  an  out- 
ward rotation  of  the  entire  leg  oc- 
curs. These  cases  are  often  called 
"saber  legs."  If  genu  varum  is 
.added  to  this  the  so-called  "O 
legs"  are  formed,  the  foot  assum- 
ing on  this  account  in  some  cases 
the  varus  and  in  others  the  val- 
gus position.  In  "X  legs"  the 
valgus  position  exists  in  both  the 
knee  and  ankle-joints.  In  the  so-called  "  baker's  legs"  one  of  the  extrem- 
ities assumes  the  valgus  position  while  the  other  remains  almost  straight. 
In  rare  cases  genu  varum  is  found  on  one  side  and  genu  valgum  on  the 
other.  In  their  longitudinal  growth  the  lower  extremities  are  more 
frequently  adversely  influenced  by  racWtis  than  are  the  upper. 

The  osseous  deformities  of  rachitis  appear  in  a  very  definite  sequence 
as  to  time.     Only  in  exceptional  cases  can  distinct  chnical  symptoms 


i. 

^B  1 

i 

„„? '      '^■^1 

Ilachitic  X-shaped  legs. 


RACHITIS  205 

referable  to  the  bones  be  noted  before  the  .end  of  the  third  month. 
Until  about  the  seventh  month  one  finds  chiefly  craniotabes  and  the 
"rachitic  rosary,"  which  corresponds  to  the  more  marked  growth  of  the 
skull  and  thorax  at  this  time.  During  the  second  half  year  craniotabes 
very  frecjuently  increases  in  extent  and  intensity.  It  is  especially  at  this 
time  that  thoracic  rachitis  becomes  prominent.  The  ribs  become  weak, 
the  rosary  often  becomes  very  marked,  and  the  deformity  of  the  thorax 
begins.  Towards  the  end  of  the  first  year  we  not  infrequently  find  the 
kyphosis.  If  the  disease  begins  after  the  end  of  the  first  year  cranio- 
tabes usually  does  not  occur.  The  thoracic  changes  still  appear,  but  at 
this  time  the  deformities  of  the  extremities  are  most  marked.  In  addi- 
tion kyphosis  and  scohosis  are  seen,  and,  as  a  late  form  of  the  cranial 
racMtis,  the  bulging  of  the  frontal  and  parietal  eminences.  The  deform- 
ities of  the  extremities  are  especially  marked  in  those  cases  in  which 
the  children  run  about  in  spite  of  the  racliitis.  Permanent  deformities 
first  appear  as  a  rule  towards  the  end  of  the  second  year. 

In  cases  in  which  rachitis  begins  in  the  middle  of  the  first  year, 
and  remains  active  far  into  the  second  year,  the  three  different  stages 
can  be  seen  in  the  same  child.  They  pass  from  one  to  another  in  sequence 
witliout  sharp  demarcation.  In  such  cases  the  craniotabes  usually  be- 
gins to  diminsh  at  about  the  end  of  the  first  year,  or  indeed  even  earlier, 
while  the  disease  advances  in  other  parts.  While  the  cUnical  symptoms 
referable  to  the  skeleton  are  gradually  developing,  the  constitutional 
symptoms,  wth  which  the  disease  began,  usually  persist.  In  a  large 
number  of  cases  all  the  deformities  undergo  complete  resolution  after 
the  active  disease  is  cured,  but  in  very  severe  cases  disfiguring  secondary 
deformities  remain  throughout  life.  The  marked  angulations  which  re- 
sult from  faulty  union  and  consolidation  of  the  diseased  bones  are  not 
capable  of  complete  resolution.  Of  the  greatest  importance  are  those 
cases  in  which  kyphoscoHosis  remains,  or  in  women,  well  marked  pelvic 
deformities.  In  less  unfavorable  cases  the  after  effects  consist  merely 
of  irregularities  of  the  bony  structures,  of  remains  of  the  "  chicken  breast" 
and  of  anomahes  in  the  position  of  the  teeth.  In  the  mildest  of  the 
severe  cases  one  finds  in  adults,  aside  from  erosions  of  the  teeth,  merely 
an  eversion  of  the  costal  arch  with  the  presence  of  Harrison's  grooves. 

(c)  Other  Organs. — A  very  small  number  of  cases  of  racliitis  present 
a  generally  excellent  condition  of  nutrition  and  a  rosy  clear  complexion. 
Tills  is  especially  true  in  breast-fed  children  that  are  onlj-  sHghtly 
racliitic.  The  majority  of  children,  although  they  may  have  been  thriv- 
ing before  the  beginning  of  the  disease  cease  to  gain  in  weight  at  its  on- 
set, and  their  body  weight  then  remains  more  or  less  below  the  normal 
throughout  the  course  of  the  affection.  Coincidentally  the  slcin  becomes 
pale  and  flabby  and  not  infrequently  assumes  a  dirty  grayish  yellow 
cachectic  hue.     The   examination  of   the  blood    has  so    far    given   no 


206  THE    DISEASES   OF    CHILDREN 

characteristic  results.  Elevations  of  temperature  which  are  observed 
in  rachitis  are  always  seen  at  the  onset  of  complications. 

The  mental  development  is  usually  not  retarded,  except  in  very 
severe  cases  in  wliich  the  general  condition  is  much  depressed,  and  also 
in  those  children  who  by  being  unable  to  run  about  and  play  are  pre- 
vented from  learning  from  other  children.  The  typical  disposition  of 
rachitic  children,  although  it  is  by  no  means  marked  in  all  cases,  is  one 
of  irritable  ill-temper.  In  protracted  cases  with  bone  pains  the  children 
finally  greet  every  one  coming  near  them  ^vith  piercing  cries;  moderate 
cases  usually  show  evidence  of  pain  only  when  handled. 

The  muscles,  especially  of  the  lower  extremities,  may  become  extra- 
ordinarily atrophied  in  those  severe  cases  in  which  the  children  for  a 
long  time  shun  all  movement,  but  the  normal  electrical  excitabihty 
always  remains.  In  consequence  of  the  fact  that  the  thoracic  muscles 
are  involved  in  the  general  muscular  weakness  the  respirations  are 
mainly  diaphragmatic.  Continued  rapid  breathing,  frequently  with 
dilatation  of  the  nostrils  occurs  in  all  cases  of  extreme  thoracic  rachitis, 
although  no  compUcation  may  be  present  in  the  lungs  or  bronchi. 
Even  with  normal  breathing  every  inspiration  retracts  the  already 
flattened  or  even  concave  lateral  chest  walls. 

The  percussion  note  is  deep  and  loud  over  the  concave  areas;  over 
the  prominent  parts  of  the  chest  higher  and  shorter.  With  considerable 
thickening  of  the  shoulder  blades  one  can  find  almost  absolute  flatness 
in  the  supraspinous  fosste.  The  area  of  cardiac  dulness  ma}'  be  increased 
in  marked  deformity  of  the  chest  even  without  cardiac  hypertrophy 
and  the  heart  shock  can  be  felt  beyond  the  usual  boundaries.  The 
respiratory  murmur,  provided  no  pulmonary  compHcations  exist,  is 
loudest  where  the  percussion  note  is  fullest.  In  racliitis  auscultation  is 
of  far  more  value  than  percussion  for  the  establishment  of  a  diagnosis 
of  pulmonary  disease.  In  doubtful  cases  it  is  always  advisable  to  form 
one's  final  opinion  only  after  many  very  careful  exaini nations.  The 
kyphoscohotic  bowing  of  the  vertebral  column  causes  further  displace- 
ment of  the  organs  and  consequently  leads  to  further  errors  in  the  usual 
topographical  diagnosis.  With  a  severe  thoracic  rachitis  the  pulse  is 
usually    quickened. 

The  appetite  is  frequently  very  good,  particularly  for  sweet  and 
starchy  foods.  The  abdomen  is  almost  always  markedly  distended  with 
gas  and  therefore  soft  and  not  sensitive  to  pressure.  The  condition  of 
the  bowels  is  not  at  all  characteristic.  In  many  cases  constipation  ex- 
ists and  often  the  feces  show  a  striking  lack  of  the  normal  pigments. 
The  liver  may  be  depressed  by  marked  thoracic  deformity  and  may, 
upon  superficial  examination,  appear  to  be  enlarged  although  in  reality 
it  is  only  dislocated  downward;  with  severe  rachitis  occurring  in  anaemic 
children  in  poor  condition  it  is  often  truly  hypertrophied.     A  marked 


RACHITIS  207 

enlargement  of  the  spleen  is  rather  rare,  and  only  occurs  when  a  high 
grade  anannia  exists.  In  many  of  these  cases  one  has  to  deal  with  a 
compUcating  hereditary  syphiUs.  The  swelling  of  the  lymph-nodes,  which 
is  frequently  noted,  has  nothing  to  do  with  racliitis  per  ae  but  is  always 
an  evidence  of  complications. 

Time  of  Onset,  Duration  and  Course. — A  positive  case  of  con- 
genital rachitis  has  as  yet  not  been  observed.  The  so-called  fcetal 
rachitis  has  no  connection  with  true  rachitis.  In  the  great  majority  of 
cases  the  first  clinical  symptoms  in  the  bones  are  noted  only  after  the 
third  month,  as  a  rule  even  in  the  second  half  year.  After  the  beginning 
of  the  second  year  the  number  of  recent  cases  diminishes,  and  the  dis- 
ease only  exceptionally  develops  after  the  end  of  the  second  year. 

It  usually  lasts  for  several  months;  often  more  than  a  year,  some- 
times more  than  two  years.  An  acute  form  of  rachitis  is  not  recognized. 
Very  frequently  in  an  individual  case  certain  of  the  very  numerous 
cHnical  symptoms  are  absent,  and  at  any  stage  of  the  disease  its  develop- 
ment may  cease;  especially  is  this  true  of  craniotabes  which  often  heals 
without  the  development  of  any  further  rachitic  symptoms.  The  first 
e\'idence  of  a  beginning  recovery  is  a  lessening  of  the  general  symptoms. 
In  very  severe  and  prolonged  cases  the  children  may  remain  weakly  for 
at  least  a  year  after  the  final  cessation  of  the  rachitic  process. 

Complications. — The  laxity  of  the  abdominal  wall  and  the  atony 
of  the  intestinal  musculature,  which  is  encountered  so  frequently  in  tliis 
disease,  give  rise  to  a  tendency  to  the  occurrence  of  umbihcal  herniae. 
These  herniae  usually  remain  small  but  they  can,  especially  under  the 
influence  of  an  intercurrent  whooping-cough,  attain  to  the  size  of  plums 
or  even  larger.     Their  prognosis  is  always  favorable. 

Complicating  pulmonary  diseases  arise  very  easily  with  severe 
thoracic  racliitis.  Even  a  moderate  bronchitis  can  be  dangerous  to  Hfe 
in  this  condition.  A  prognosis  should  therefore  always  be  made  with 
care.  The  worst  feature  is  the  fact  that,  in  racliitis,  catarrh  has  a  dan- 
gerous tendency  to  descend  to  the  finer  bronchioles,  especially  in  poorly 
nourished  individuals,  and  thus  to  lead  to  capillary  broncliitis  and 
bronchopneumonia.  The  prognosis  for  the  last  two  diseases  is  always 
grave  in  rachitic  children. 

Diseases  of  the  gastro-intestinal  canal  are  frequent  and  serious 
complications.  Especially  to  be  feared  are  those  catarrhal  diseases  of 
the  large  intestine  which  are  accompanied  with  mucus  stools.  In  the 
case  of  debiUtated  children  the  associated  intestinal  catarrh  frequently 
becomes  the  specific  cause  of  death. 

The  nervous  complications  are  very  important,  especially  spasm 
of  the  glottis,  eclampsia  and  tetany.  The  importance  of  these  spasms 
lies  in  the  danger  of  sudden  death  in  a  laryngospastic  or  eclamptic 
attack.      Sometimes    nystagmus,    rotatory   spasm   or    spasmus   nutans 


208  THE   DISEASES   OF   CHILDREN 

occur.  The  prognosis  of  these  forms  of  spasm  is  good,  and  recovery 
almost  always  ensues  after  a  few  months  of  antiracliitic  treatment. 
The  occasional  occurrence  of  cataleptic  conditions  in  poorly  nourished 
rachitic  cliildren  should  be  noted.  The  symptoms  are  those  of  the  well 
marked  "  Flexihilitas  cerea."  Finally,  the  fact  can  hardly  be  disputed, 
that  children  with  severe  racliitis  are  more  disposed  than  others  to 
tuberculosis  of  the  various  organs. 

Pathological  Anatomy. — According  to  the  severity  of  rachitis,  the 
anatomical  changes  are  of  vastly  different  grades,  from  the  very  light 
manifestations,  whose  differentiation  from  physiological  states  is  diffi- 
cult, to  the  severest  lesions.  In  all  cases  except  the  mildest  the  rigidity 
of  the  bones  is  markedly  impaired;  occasionally  this  advances  so  far 
that  one  can  bend  the  bones  as  if  they  were  made  of  rubber.  At  the 
same  time  there  exists  a  considerable  hypericmia  of  the  periosteum'  and 
marrow.  The  periosteum  is  more  or  less,  often  very  markedly,  thick- 
ened; the  thickening  being  due  almost  entirely  to  an  increase  of  the 
cambium  layer.  Directly  under  the  fibrous  layer  are  found  uncalcified 
spicules  of  young  bony  tissue  formed  by  ossification  of  the  periosteum; 
between  them  lie  the  primary  periosteal  marrow  spaces.  In  the  deeper 
layers  of  the  swollen  cambium  the  new  formed  bony  spicules  are  stronger, 
and  the  spaces  between  them  are  narrower.  The  spicules  which  were 
first  formed  contain  calcified  fragments  in  their  central  portions  but  con- 
sist otherwise  almost  entirely  of  uncalcified  osteoid  tissue.  Further  on 
one  comes  to  the  cortex  proper  into  which  open  narrower  vessel  canals 
than  the  normal  qanaliculi.  The  lamella  of  the  cortex  everywhere  show 
abnormally  broad  osteoid  borders.  Only  in  the  places  where  the  bony 
tissue  is  undergoing  absorption,  wliich  is  especially  apt  to  be  of  greater 
extent  in  the  neighborhood  of  the  central  marrow  cavity,  does  the  cal- 
cified bony  tissue,  which  is  well  supplied  with  lucunar  spaces,  border 
directly  upon  the  marrow  tissue. 

Fractures  occur  most  easily  at  the  time  at  which  the  new  and  pro- 
portionately soft  deposits  form  a  fairly  thick  layer  upon  the  deeper, 
compact,  and  relatively  tliin  bony  sheath  which  surrounds  the  central 
marrow  cavity.  The  breaks  in  continuity  occur  for  the  most  part  only 
in  the  compact  layer  while  the  young  superficial  layers  are  compressed. 
Breaks  occur  usually  only  in  the  concave  portions  which  he  in  the  angle  of 
fracture,  .similar  to  the  breaks  which  occur  during  the  forcible  bending 
of  a  willow  twig  (green-stick  fracture),  or  a  feather  quill.  The  frag- 
ments are  displaced  towards  the  convex  side  wliich  narrows  and  often 
entirely  closes  the  marrow  canal;  the  marrow  is  accordingly  pressed 
out  and  in  part  destroyed. 

Callus  formation  occurs  in  every  place  where  the  bone  is  fractured 
and  usually,  too,  for  a  considerable  distance  about  the  break.  Some- 
times the  entire  angle   of  fracture  is  filled   with  callus  winch  usually 


RACHITIS 


209 


forces  itself  into  the  marrow  cavity  through  the  aperture  of  the  fracture 
and  thus  leads  to  a  complete  closure  of  the  cavity  at  this  point.  A  con- 
tinuous marrow  ca\'ity  is  reformed  only  a  very  long  time  after  consolida- 
tion. Racliitic  callus  bears  a  close  resemblance  to  the  young  periosteal 
growth  except  that  it  far  more  frequently  contains  true  cartilage. 
In  the  hghtest  cases  the  periosteal  thickening  affects  only  the  edges 


Fig.  37. 


m  ' 


Longitudinal  section  inniULrii  ihe  proximal  epiphyseal  end  of  the  tibia.    Nineteeu 
year  old  boy  with  moderately  severe  rachitis. 


of  the  cranial  bones,  but  in  the  more  severe  ones  the  tuberosities  also 
are  involved.  The  inner  surface  of  the  bones  at  all  times  remains  free 
from  racliitic  deposits.  The  membranes  which  cover  the  sutures  and 
fontanelles  are  lax,  and  tliickened.  The  cranial  bones  are  never  all 
equally  affected  by  rachitis;  in  typical  cases  the  frontal  bones  are  only 
shghtly  thickened  at  the  edges,  while  the  parietal  bones  are  partly,  and 
the  occipital  almost  entirely,  covered  by  a  red  spongy  deposit.  Very 
11—14 


210  THE   DISEASES   OF   CHILDREN 

frequently  there  occurs  at  the  same  time  in  the  region  of  the  occiput  a 
tliinning  of  the  bony  plates  which  in  severe  cases  amounts  to  the  ap- 
pearance of  membranous  gaps.  Such  a  membranous  area  always  cor- 
responds \vith  a  digitate  (Pacchionian)  depression  of  the  inner  surface, 
while  on  the  outer  side  its  upper  surface  Hes  on  the  same  level  as  the 
surrounding  tissue.  In  all  these  cases  of  craniotabes,  the  other  bones, 
especially  frequently  the  ribs,  show  macroscopic  rachitic  changes,  and 
with  microscopic  examination  one  never  fails  to  find  osteoid  tissue  in 
these  craniotabetic  areas.  As  to  the  remaining  flat  bones,  the  only 
point  to  be  emphasized  here  is  the  fact  that  the  pelvic  bones  very  early 
undergo  the  well-known  changes  which  are  so  important  in  obstetrics; 
but  apparently  in  the  pelvis  as  well  as  elsewhere  the  rachitic  lesions  are 
in  many  cases  either  completely  or  partially  resolved  in  the  course  of 
further  growth. 

The  disturbances  wliich  endochondral  ossification  undergoes  in 
rachitis  are  very  characteristic.  As  a  result  of  the  delay  in  the  ossifi- 
cation caused  by  the  advancing  growth  of  the  cartilage  cells,  the  pri- 
mary growing  margin  assumes  an  abnormal  width.  Later  on  provisional 
cartilaginous  calcification  occurs  but  still  only  in  an  incomplete  man- 
ner; then,  through  further  cartilaginous  growth,  the  formation  of  the 
marrow  cavity  advances  irregularly  over  the  fine  of  ossification,  and 
finally,  by  a  continuation  of  the  above  phenomena,  narrow  marrow 
cavities  are  formed  in  the  cartilage,  the  edges  of  wliich  undergo  a  meta- 
plasia into  osteoid  tissue.  In  all  stages  the  newly  formed  bony  tissue 
remains  more  or  less  completely  uncalcified  in  the  neighborhood  of  the 
line  of  ossification  as  it  does  elsewhere  in  the  skeleton.  With  a  continua- 
tion of  the  disease  these  phenomena  always  become  more  striking. 

Very  frequently  the  rachitic  bones  show  a  more  or  less  marked 
degree  of  osteoporosis,  in  addition  to  the  changes  which  have  been 
above  described.  The  consistency  of  the  bones  is  probably  impaired 
rather  by  a  marked  osteoporosis  than  by  the  persistence  of  the  newly 
formed  bone  substance  in  an  osteoid  condition.  After  the  complete 
cessation  of  the  rachitic  process  the  bones  attain  an  abnormal  thickness 
\\ith  a  correspondingly  increased  hardness,  and  since  they  are  tliicker 
than  normal  there  results  a  considerable  increase  in  their  weight.  The 
apophyses  and  the  other  muscle  attachments  are  unusually  strongly 
marked,  the  normally  sharp  edges  are  rounded  off,  and  their  whole 
appearance  is  coarse  ■  and  unsymmetrical.  The  erosions  on  the  teeth 
remain  throughout  life. 

Of  the  lesions  in  the  soft  tissues  only  the  rather  frequent  occurrence 
of  extensive  enlargements  of  the  ventricles  of  the  brain  needs  mention. 

Pathological  Chemistry. — The  water  content  of  rachitic  bones  is 
higher  than  normal;  in  the  fat  content  there  are  no  constant  differences. 
The  most  important  chemical  property  of  rachitic  bones  is  the  decrease 


RACHITIS 


!211 


of  mineral  constituents,  especially  calcium  and  phospiioric  acid.  The 
percentage  of  ash  in  the  ribs  and  vertebra;  is  especially  low,  often  only 
25  per  cent.,  or  less,  of  the  normal.  The  specific  gravity  of  the  cartilages 
and  the  bones  is  decreased  and  the  relative  weight  of  the  corte.x  of  the 
spongiosa  and  of  the  cartilages  i.s  changed  in  favor  of  tlie  cartilage. 
The  soft  tissues  of  rachitic  children  contain  no  less  calcium  than  normal. 
The  urine  is  usually  faintly  acid;  during  the  active  stage  of  the  disease 
its  calcium  content  is  usually  somew^iat  reduced  wliile  the  percentage 
of  calcium  in  the  feces  at  tliis  time  is  always  shghtly  increased. 

Relation  of  Rachitis  to  Osteomalacia;  Late  Rachitis. — The   causes  of 
rachitis  and  of  osteomalacia  are  without  doutit  distinct.     The  patholog- 

Fi(i.   .38. 


ical  processes  in  the  bone  are,  however,  in  a  manner  the  same.  In  osteo- 
malacia the  disturbance  of  endochondral  ossification  is  less  marked  than 
the  occurrence  of  osteoporosis,  but  this  difference  is  explained  bj'  the 
diver!5ity  in  the  ages  of  the  patients.  The  marked  o.steoid  deposits  in 
the  bones  in  osteomalacia  consist  of  new  formed  uncalcified  lione  tissue; 
in  rachitis,  on  the  contrary,  there  occurs  in  restricted  areas  supplemen- 
tary decalcification  of  already  normally  calcified  bone.  Osteomalacia, 
therefore,  from  a  pathological, — not  an  etiological, — standpoint,  may  be 
considered  as  a  rachitis  of  later  life.  The  tran.sition  forms  which  occur 
in  young  adults,  over  the  classification  of  which  as  rachitis  or  osteo- 
malacia there  has  heretofore  been  mucli  argument,  present  no  difficul- 


212  THE    DISEASES   OF   CHILDREN 

ties  with  tliis  perception  of  tlieir  relation.  In  the  cases  wliich  have  been 
described  as  infantile  osteomalacia,  and  wliich  curiously  always  occurred 
in  girls,  racMtis  resembles  remarkedly  in  its  skeletal  phenomena  the 
otherwise  characteristic  behavior  of  osteomalacia  of  later  life.  These 
cases  of  rachitis  are  in  marked  contrast' to  those  in  wliich  disturbances 
of  cartilaginous  ossification  are  the  most  prominent  manifestations  of 
the  disease  (von  Recklinghausen's  "pure  racliitis"). 

According  to  the  opinion  of  authoritative  surgeons  the  static  de- 
formities which  appear  at  the  time  of  puberty  develop  upon  the  founda- 
tion of  true  racliitis.  There  are  in  such  cases  specific  racliitic  ciianges 
in  the  epiphyseal  cartilages,  and  to  a  greater  or  less  extent,  periosteal 
osteophytic  formation  is  also  found. 

Etiology  and  Pathogenesis. — The  cause  of  rachitis  is  unknown. 
Siegert  has  again  recently  drawn  emphatic  attention  to  the  occurrence 
of  hereditary  predisposition  to  the  affection  wliich,  according  to  his 
theory,  is  transmitted  principally  by  the  mother.  Cliildren  with  this 
tendency  are  not  protected  by  maternal  nursing  but  in  such  cases  the 
disease  is  usually  mild.  Only  exceptionally  does  severe  racliitis  occur 
in  breast-fed  children.  If  tliis  hereditary  tendency  is  absent  .children 
often  remain  free  from  the  disease  even  if  fed  artificially.  Racliitis  has 
no  relation  to  herecUtary  sypliilis. 

Many  explanations  are  offered  for  tlie  nature  and  pathogenesis  of 
rachitic  bone  processes.  The  decalcified  remains  of  the  new  formed  bone 
tissue  finally  reach  the  stage  at  wliich  they  are  unable  to  absorb  the  lime 
salts  that  are  abundantly  present  for  their  use.  Only  in  this  one  respect 
is  the  calcium  metabohsm  disturbed  by  rachitis;  beyond  a  certain  point 
the  question  is  as  httle  one  of  deficient  calcium  supply  as  it  is  of  defi- 
cient calcium  absorption. 

It  is  true  that  if  an  experimental  diet  very  poor  in  calcium  is  given 
to  quickly  growing  young  animals,  it  leads  to  a  disease  which  seems, 
upon  superficial  examination,  to  be  extraordinarily  similar  to  rachitis. 
Histological  examination,  however,  shows  that  here  we  have  to  deal  not 
with  true  racliitis,  but  with  an  osteoporosis  with  rachitic-like  changes 
in  the  periosteum  and  in  the  growing  cartilages.  With  this  pseudo- 
racliitic  osteoporosis,  in  contrast  to  rachitis,  the  soft  tissues  also  take 
part  in  the  calcium  deficiency.  Furthermore  Pfaundler  has  very  re- 
cently proved  in  a  briUiant  manner  that  racliitic  osteoid  tissue  is  not 
able  to  correct  its  calcium  deficiency  by  means  of  absorption  from  a 
solution  of  calcium  chloride,  but  that  in  the  pseudorachitic  osteoporosis 
of  animals  fed  with  a  diet  deficient  in  calcium  the  calcium  absorption  is 
markedly  increased. 

Racliitis  can  also  be  experimentally  produced  by  the  feeding  of 
acids  in  the  same  way  as  with  a  diet  deficient  in  calcium. 

The  hypotheses  according  to  which  rachitis  is  due  to  a  lessening  of 


RACHITIS  i\S 

the  alkalinity  of  the  blood,  to  the  accumulation  of  lactic  acid  in  the 
organism,  or  to  the  retention  of  abnormally  large  amounts  of  carbonic 
acid,  are  as  equally  erroneous  as  the  one  last  considered,  which  assumes 
a  primary  disturbance  in  the  calcium  metabohsm. 

In  the  majority  of  cases  rachitis  develops  in  the  colder  part  of  the 
year  in  which  tlie  children  verj^  seldom  go  out  of  doors,  just  as  it  never 
occurs  in  wild  animals  in  their  free  state,  and  very  seldom  in  pastured 
animals,  but  very  frequently  in  animals  in  stables  and  zoological  gar- 
dens. Perhaps  further  investigations  into  these  observ'ations  ma}'  some 
day  lead  to  the  elucidation  of  the  origin  of  tWs  curious  disease. 

Diagnosis. — Before  the  appearance  of  the  characteristic  skeletal 
changes  the  diagnosis  of  racliitis  can  be  only  presumptively  made.  In 
the  newborn  there  occurs  not  infrequently  a  defective  ossification  of 
the  flat  cranial  bones  which  can  be  confused  with  craniotabes,  but  it 
may  be  distinguished  from  the  latter  by  the  less  marked  limitation  of 
the  depressible  areas  to  the  occipital  bones,  as  well  as  by  its  course,  as 
it  reaches  its  full  development  in  the  first  week  or  month  after  birth, 
while  true  craniotabes  never  occurs  congenitally  and  usually  appears 
first  after  the  end  of  the  third  month  of  life.  Furthermore,  according 
to  the  investigations  of  Friedlebcn,  there  exists  in  all  children  during  the 
second  three  months  of  Ufe  a  physiological  increase  of  the  bony  absorp- 
tion in  the  flat  cranial  bones  which  can  be  the  more  easily  mistaken  for 
craniotabes  as  it  affects  principally  the  posterior  part  of  the  skull.  It 
is  more  frequent  in  artificially  fed  cliildren  than  in  those  fed  from  the 
breast,  and  is  clinically  manifested  by  the  fact  that  the  posterior  part 
of  the  cranial  vault  becomes  flexible  and  compressible  ;  on  careful 
palpation,  however,  one  always  finds  a  feeling  of  complete  elastic- 
ity of  the  bony  plates  while  in  rachitic  craniotabes  the  affected 
areas  have  more  or  less  completely  lost  their  elasticity;  they  are  not 
flexible  but  soft.  Without  other  symptoms  a  diagnosis  of  rachitis 
cannot  be  made  from  the  time  of  the  appearance  of  the  first  teeth,  for 
in  non-rachitic  children  they  often  appear  first  in  the  9th  or  even  in 
the  11th  month. 

The  softening  of  the  thorax  is  shown  by  the  retraction  at  each 
inspiration,  and  is  most  distinct  with  quiet  breathing,  but  one  should 
remember  that  in  young  children  without  racliitis  the  thorax  is  far 
more  elastic  than  in  later  years,  and  that  a  corresponding  suggestion  of 
inspiratory  retraction  occurs  even  in  absolutely  normal  infants.  For 
an  early  diagnosis  the  occurrence  of  the  racliitic  rosary  is  far  more  im- 
portant than  the  pliability  of  the  thorax.  Still  it  is  to  be  emphasized 
that  slight  swelling  at  the  costal  end  of  the  costal  cartilages  is  frequently 
present  even  without  rachitis.  Naturally  no  sharp  distinction  can  be 
made  between  these  shght  enlargements  and  the  somewhat  larger  ones 
which    are   referable    to   rachitis.    The  same  thing  holds  good  for  the 


214  THE   DISEASES   OF   CHILDREN 

epiphyses  of  the  long  bones;  here  too  there  is  a  gradual  transition 
between  the  pathological  and  physiological  enlargements. 

The  early  chfferentiation  of  racliitis  from  infantile  myxoedema  is 
very  important.  Myxoedematous  children  also  present  swellings  on  the 
costal  cartilages  and  tliickenings  of  the  epiphyses  of  the  long  bones,  and 
with  them  even  more  than  in  rachitis  there  is  a  delay  in  the  appearance 
of  the  teeth,  as  well  as  in  the  closure  of  the  larger  fontanelle,  and  in 
learning  to  stand  and  walk.  Nevertheless  such  a  striking  clinical  pic- 
ture is  presented  by  the  combination  of  a  marked  delay  in  the  growth, 
with  a  considerable  retardation  of  the  psychic  development,  cretinoid 
physiognomy,  a  myxoedematous  condition  of  the  subcutaneous  fatty 
tissue,  and  \vith  macroglossia,  that  an  experienced  individual  can  easily 
make  a  correct  diagnosis  at  the  first  glance  even  in  the  less  marked 
cases.  Furthermore  in  rachitis  the  skin  is  soft  and  delicate;  in  myx- 
oedema  it  is  dry  and  hard,  often  vnih  mucii  thickened  epidermis,  espe- 
ciallj'  of  the  toes;  the  hair  in  racliitis  is  tliin  and  soft,  in  myxoedema 
coar-se  and  brittle;  most  racliitic  children  sweat  a  great  deal,  wliile 
as  a  rule  myxoedematous  ones  do  not;  finally  the  poor  appetite  and 
extreme  constipation  of  myxoedema  are  features  which  never  occur  in 
rachitis  in  such  marked  degree. 

The  confusion  of  infantile  scurvy  (Barlow's  disease)  and  rachitis 
arises  from  the  fact  that  the  former  malady  was  earlier  considered  to  be 
the  same  as  acute  or  htemorrhagic  rachitis.  For  the  establishment  of  a 
correct  diagno.sis  it  should  be  noted  that  infantile  scurvy  preferably 
affects  rachitic  children,  and  that  the  occurrence  of  undoubted  rachitic 
symptoms  in  no  way  excludes  or  modifies  those  of  the  other  disease. 
Properly  speaking  the  two  diseases  have  only  one  symptom  in  common, 
namely  the  tenderness  of  the  bones,  and  even  this,  especially  in  the 
legs,  is  apt  to  be  of  such  extraordinary  severity  in  infantile  scurvy  as  is 
only  very  seldom  seen  in  rachitis.  The  swellings  on  the  bones  in  infan- 
tile scurvy,  which  are  caused  by  subperiosteal  hsematomata,  are  easily 
differentiated  from  the  epiphyseal  enlargements  of  rachitis  by  their 
locaUzation,  as  they  affect  not  the  epiphyses  themselves  but  the  neigh- 
boring portion  of  the  diaphyses.  There  is  moreover  in  the  symptoma- 
tology of  racliitis  notliing  wliich  one  could  at  all  confuse  with  the  other 
symptoms  of  infantile  scurvy,  namely,  the  affection  of  the  gums,  and 
the  haemorrhages  into  the  eyelids  and  orbits,  as  well  as  the  other  indica- 
tions of  the  h;pmorrhagic  diathesis. 

Hereditary  syphihs  can  enter  into  the  differential  diagnosis  of 
rachitis  in  manifold  ways.  Among  others  the  syphilitic  pseudoparalysis 
of  Parrot  is  confused  with  it,  but  the  locahzation  of  the  bony  enlarge- 
ments differs  in  the  two  diseases.  In  rachitis  the  swelling  preferably 
affects  the  epiphyses;  in  syphihtic  pseudoparalysis  there  occurs  either 
a  thick  ridge  which  surrounds  the  bone  just  at  the  epiphyseal  margin, 


RACHITIS  215 

or  a  spindlc-liko  swelling  which  involves  both  the  epiphysis  and  the  end 
of  the  diaphysis.  A  further  differential  point  is  the  complete  flaccid 
pseudoparalysis  which  gives  the  disease  its  name,  and  which  often  re- 
mains a  prominent  symptom,  during  the  entire  course  of  the  affection. 
The  pseudoparalysis  affects  the  lower  epiphysis  of  the  humerus  with 
special  predilection;  still  this  point  should  not  be  very  much  empha- 
sized, as  the  distal  epiphysis  of  the  radius  and  indeed  the  bones  of  the 
lower  extremities  are  often  involved.  More  important  is  the  fact  that 
the  syphihtic  paralysis  is  very  often  unilateral  or  at  least  is  more  pro- 
nounced on  one  .side,  wliile  the  epiphyseal  enlargements  due  to  rachitis 
are  almost  always  completely  symmetrical;  and  in  further  contrast  to 
racliitis  the  pseudoparalysis  affects  only  one  joint  or  at  most  a  few. 
Lastly  the  time  of  onset  of  the  swelUng  is  very  important.  The  pseudo- 
paralysis is  a  disease  mostly  of  the  first  three  months  of  Ufe  wliile  the 
epiphyseal  enlargement  of  rachitis  usually  occurs  at  a  later  time.  In 
rare  cases  the  tibial  deformity  due  to  racliitis  resembles  the  "sabre 
blade"  form  wliich  is  characteristic  of  syphilis,  but  usually  the  laterally 
bent  rachitic  tibia  can  be  differentiated  at  the  first  glance  from  that 
due  to  syphihs,  which  is  laterally  compressed  and  bent  directly  forward. 
In  the  rare  doubtful  cases  the  racliitic  origin  of  the  deformity  is  improb- 
able if  no  such  marked  deformities  are  found  elsewhere  in  the  body. 
Furthermore  it  should  be  again  emphasized  that  a  combination  of 
rachitis  and  syphilis  is  possible. 

A  marked  rachitic  prominence  of  the  frontal  and  parietal  tuber- 
osities can  so  strikingly  exceed  the  facial  portion  of  the  skull  as  to  sug- 
gest the  occurrence  of  hydrocephalus,  but  in  the  latter  condition  the  ab- 
normal expansion  affects  the  entire  skull  more  symmetrically,  especially 
the  lateral  areas  lying  below  the  tubera.  Besides  this  the  bulging  and 
tension  of  the  large  fontanelle  and  the  separation  of  the  sutures  are  im- 
portant symptoms.  The  position  of  the  eyeballs  in  hydrocephalus  is 
absolutely  characteristic;  they  are  rotated  downward  so  that  the  lower 
and  not  the  upper  part  of  the  iris  is  hidden  beneath  the  eyelid  and 
between  the  upper  margin  of  the  iris  and  the  upper  eycHd  a  strip  of 
sclera  is  frequently  vi.sible. 

The  differentiation  may  be  very  difficult  if  hydrocephalus  begins 
to  develop  in  a  rachitic  child.  Often  in  such  circumstances  only  the 
further  course  of  the  disease  can  bring  a  sure  diagnosis. 

The  sharply  localized  tuberculous  kypho.sis  which  cannot  be  re- 
duced without  the  use  of  force  may  be  in  almost  all  cases  very  easily 
differentiated  from  the  Hke  deformity  due  to  rachitis,  as  the  latter  al- 
ways involves  many  vertebrae,  in  a  gentle  curve  rather  than  an  acute 
angle,  and  can  be  partially  or  entirely  reduced  by  raising  the  patient's 
legs  when  he  lies  in  a  prone  position.  The  congenital  "Trichterbrust," 
or  Funnel  Chest,  differentiates  itself  from  the  similar  deformitv  due  to 


216 


THE    DISEASES   OF   CHILDREN 


rachitis  by  the  tremendous  retraction  of  the  inferior  end  of  the  sternum. 
Rachitic  coxa  vara  can  simulate  congenital  dislocation  of  the  hip  by  the 
high  position  of  the  trochanter  major,  and  by  the  waddling  gait.  Other 
marked  rachitis  deformities  usually  point  at  once  to  the  right  diagnosis 
and  in  doubtful  cases  radioscopy  throws  a  deciding  light  upon  the  subject. 
As  for  the  erosions  of  the  teeth,  the  Hutcliinsonian  semilunar 
Fig.  39.  defccts  of  the  edges  and  the 

rounding  off  of  the  corners 
of  the  upper  central  incisors, 
wliile  at  the  same  time  these 
teeth  are  shortened  and  di- 
minished in  size  and  are  so 
placed  that  the  cutting  edges 
converge,  must  always,  even 
in  the  slight  cases,  arouse  a 
very  strong  suspicion  of  hered- 
itary sypliihs.  The  other 
forms  of  erosions  so  far  as 
they  affect  the  incisors  and 
the  first  molars  are  due  to  a 
long  standing  rachitis. 

Frequently  such  a  high 
grade  of  atrophy,  flaccidity 
and  inactivity  of  the  muscu- 
lature, especially  of  the  lower 
extremities,  is  met  with,  that 
it  is  not  possible,  without  other 
symptoms,  to  exclude  mth  cer- 
tainty a  paralysis  due  to  ante- 
rior poliomyelitis.  In  such  cases 
an  examination  of  the  electri- 
cal reactions  of  the  affected 
muscle-groups  is  necessary. 

In  especially  marked  ra- 
chitic meteorism  with  disten- 
tion of  the  abdomen  the  differentiation  from  tuberculous  peritonitis 
may  be  very  difficult  if  the  cliild  is  elsewhere  possibly  tuberculous; 
for  indistinct  dulness  may  be  present  in  the  dependent  portions  of  the 
abdomen  in  both  diseases,  and  the  bowel  movements  may  present  the 
same  greasy  consistence,  the  same  striking  light  color  and  the  same 
very  intense  odor.  In  such  cases,  especiall}'  in  the  ab.sence  of  fever,  the 
examination  of  the  urine  for  the  diazo  reaction  is  of  value.  In  other 
cases  the  differentiation  between  the  conditions  mentioned  can  be  safely 
made  only  after  long  observation. 


■ 

^^B 

HI^HI 

/ 

"'*  ^^'{^^^^^^^^^^^^^^^^^i 

^^'  ■'"/' 

1 

m 

1 

^^^^^L^-' 

,  J 

'^ 

Rachitic  funnel-breast. 


RACHITIS  in 

Prognosis. — The  light  and  medium  cases  of  rachitis  all  progress  to 
complete  recovery  provided  they  remain  free  from  serious  complica- 
tions, and  fall  soon  enough  into  expert  hands  for  treatment.  The 
warmer  part  of  the  year  is  more  suitable  for  recovery  from  rachitis  than 
the  cold.  The  majoi-ity  of  the  severe  deformities  disappear  gradually 
during  cliildhood;  only  in  very  severe  cases  do  considerable  disfigure- 
ments remain  throughout  hfe.  In  the  most  severe  cases  rachitic  dwarf- 
ing may  result.  The  danger  of  a  persisting  scoUosis  lies  in  the  fact  that 
static  influences  may  increase  the  deformity  even  after  the  cessation  of 
the  active  racliitic  process.  Malformations  of  the  thorax  and  spine  may 
lead  in  later  years  to  cardiac  insufficiency,  and  malformations  of  the 
pelvis  to  obstetrical  difficulties. 

Prophylaxis. — With  our  present  knowledge  racliitis  cannot  with 
surety  be  prevented;  but  careful  dietetic  management  is  very  valuable 
and  the  earUest  possible  antirachitic  treatment  is  very  important. 
The  disease  seldom  takes  a  severe  hold  upon  breast-fed  children  who 
live  in  dry  sunny  rooms,  who  are  much  in  the  fresh  air  and  are  bathed 
frequently.  With  artificially  fed  children  an  important  point  is  the  pre- 
vention of  overfeeding.  Children  with  severe  thoracic  rachitis  should 
be  carefully  protected  from  whooping-cough,  measles  and  influenza.  If 
severe  softening  of  the  bones  exists,  the  occurrence  of  multiple  fractures 
should  be  guarded  against  by  encasing  the  hmbs  in  stiff  dressings. 

Therapy. — The  best  therapeutic  agent  for  rachitis,  according  to 
my  opinion,  is  codliver  oil  with  phosphorus.  Tliis  always  acts  especially 
quickly  upon  the  general  symptoms  and  upon  the  dangerous  spasm  of 
the  glottis,  a  feature  which  shows  an  extraordinary  superiority  and  one 
which  no  other  antiracliitic  remedy  shares  with  it.  It  is  best  begun 
with  the  dose  of  i  teaspoonful  daily  of  the  usual  solution  [phosphorus 
.01  Gm.,  codUver  oil  100  Gm.  [phosphorus  gr.  |  and  codliver  oil  Siii]  and 
gradually  increased  to  4  teaspoonful  twice  a  day.  With  this  method  of 
exhibition  it  usually  agrees  well  with  the  patient.  Codliver  oil  mthout 
the  phosphorus  is  also  a  valuable  remedy.  One  begins  by  giATng  a  scant 
teaspoonful  increasing  the  dose  gradually  to  about  three  tea.spoonfuls 
daily.  It  is  best  taken  at  meal  time  because  it  is  then  acted  upon  by  the 
richly  secreted   pancreatic  juice  at  the  same  time  with  the  other  food. 

The  diet  of  racliitic  children  has  to  be  watched  carefully  in  order 
that  overfeeding  may  be  avoided.  With  artificial  feeding  it  is  advisable 
to  add  some  fresh  fruit  and  vegetables  to  the  other  food  even  from  about 
the  seventh  month,  and  to  give,  in  adtUtion  to  the  cow's  milk,  meat 
broth,  yolk  of  egg,  fresh  scraped  mutton,  fresh  vegetable  piu-ee,  fresh 
fruit  juice  and  the  like.  Naturally,  all  these  articles  ai'e  to  be  given 
only  in  small  amounts,  and  only  once  a  day.  In  order  to  increase  the 
diet  of  thin  racliitic  cliildren  a  small  teaspoonful  of  malt  extract  may 
be  given  two  or  three  times  a  day,  either  clear  or  stirred  in  the  milk. 


218  THE   DISEASES   OF   CHILDREN 

Salt  baths  for  fat,  pasty  cliildren  act  well,  but  wth  thin  erythema- 
tous ones  they  do  more  harm  than  good.  They  should  be  given  two  or 
three  times  a  week  for  about  10  minutes  at  a  time  at  about  33°  C.  (93° 
F.),  and  containing  1  to  4  pounds  (h  to  2  kilos)  of  sea  salt  to  a  bath. 

Residence  at  the  seashore  is  valuable  as  a  climatic  cure  but  for 
various  reasons  it  is  only  exceptionally  available.  In  cases  in  which 
there  is  no  special  tenderness  it  is  advisable  to  employ  regular  bathing 
\\ith  alcohohc  Hquids,  or  gentle  rubbing  with  aromatic  liniments.  After 
the  decUne  of  the  active  stage,  massage  properly  carried  out  for  a  month 
or  so  acts  very  favorably  upon  the  general  health,  the  abihty  to  walk, 
and  even  upon  the  growth. 

The  Epstein  rocking  chair  is  especially  recommended  for  children 
with  beginning  spinal  deformity.     With  severe  craniotabes  it  is  well  to 

P'iG.  40. 


Epstein  rocking  chair. 

have  the  head  of  the  child  lie  upon  a  soft  ring  or  upon  a  horse  hair 
pillow  with  a  depression  in  the  centre. 

Angular  deformities  of  the  long  bones  are  best  corrected  in  the 
active  stage  of  the  disease,  but  it  is  far  more  judicious  to  delay  the  or- 
thopedic treatment  of  rachitic  deformities  of  the  hmbs  until  the  sixth 
or  seventh  year.  Very  frequently  even  severe  deformities  correct  them- 
selves spontaneously  before  this  time  if  the  child  grows  strong.  In  the 
case  of  racliitic  dwarfs  a  long  continued  and  careful  treatment  with 
small  doses  of  thyroid  extract  is  beneficial,  in  addition  to  the  massage. 
The  treatment  of  rachitic  fractures  of  the  femur  by  vertical  extension 
is  to  be  condemned,  for  rachitic  bones  wliich  are  vertically  suspended 
undergo  an  acute  softening,  the  anatomical  basis  of  which  is  an  acute 
rachitic  osteoporosis. 


PLAIK   10. 


DOUBLE   RACHITIC   COXA  VARA. 

(4-year-ol(l   girl) 


DIABETES  MELLITUS 

1)Y 

Professoii  C.    vox  NOORDEX,  of  Vienna 

translated  hy 
Dr.  ANDREW  MACFARLANE,  Albany,  X.  Y. 


Diabetes  Mellitus  was  formerly  regarded  as  a  very  rare  disease 
in  childhood.  Tliis  beUef  is  not  entirely  correct  as  a  great  number  of 
cases  of  diabetes  in  cliildhood  have  been  reported  in  the  last  ten  years, 
due  not  to  its  increased  frequency  but  to  its  better  recognition.  Most 
statistics  show  that  from  .5  to  1  per  cent,  of  all  cases  of  diabetes  occur 
in  the  first  ten  years  of  Hfc  but  my  own  records  embracing  2000  patients 
give  2.5  per  cent,  for  the  first  decade.  The  .second  half  of  this  period  is 
more  affected  than  the  first,  although  the  earhest  infancy  is  not  entirely 
exempt  from  this  disease.  Many  ca.ses  at  tliis  early  age  are  probably 
undetected;  indeed  many  a  child  who.se  death  certificate  has  stated 
gastro-intestinal  catarrh,  atrophy,  asthenia,  may  in  truth  have  died  from 
diabetes. 

It  is  therefore  not  superfluous  to  advise  that  the  examination  of  the 
urine  even  in  the  earliest  cliildhood  lie  not  neglected.  Whoever  regularly 
examines  the  urine  of  young  children,  will  often  be  astounded  by  the 
positive  result  of  the  test  for  sugar  and  will  be  alarmed  if  he  is  not 
cognizant  of  certain  pecuHarities  in  cliildhood.  Small  quantities  of  milk- 
sugar  may  appear  in  the  urine  of  breast-  and  bottle-fed  babies  and  espe- 
cially when  milk-sugar  is  added  to  the  bottle  milk  in  order  to  overcome 
constipation  or  to  improve  the  nutrition.  This  alimentary  lactosuria  is 
naturally  of  no  importance.  Milk-sugar  may  be  identified  by  the  yel- 
lowish red  or  brownish  precipitate  in  Rubner's  copper  test  instead  of 
the  cherry  red  color  due  to  grape-sugar.  The  fermentation  test  is  nega- 
tive when  the  urine  has  been  prcviou.sly  sterilized  by  heat.  The  best 
method  of  determination  is  to  inoculate  the  urine  vdth  a  piu^e  culture  of 
saccharomyces  apiculatus:  if  grape-sugar  is  present  there  is  marked 
fermentation,  wliich  is  absent  ^^^th  milk-sugar. 

Young  children  show  a  much  greater  tendency  to  transitory  glycos- 
uria than  do  adults.  In  severe  diphtheria  and  especially  in  pneumonia 
with  liigh  fever  the  ingestion  of  moderate  quantities  of  carbohydrates 
may  induce  a  glycosuria,  a  resulting  condition  wliich  occurs  also  in 
adults  much  oftener  than  the  text  books  indicate.  This  is  also  transitory 
and  to  be  attributed  to  functional  changes  in  the  pancreas  due  to  the 
intoxication.  I  have  seen  this  tendency  to  glycosuria  continue  several 
days  longer  than  the  original  disease  and  in  one  case  for  two  weeks. 

219 


220  THE   DISEASES   OF   CHILDREN 

On  account  of  the  relative  frequency  of  this  undeniable  transitory 
glycosuria  in  cliildren,  the  diagnosis  of  diabetes  should  not  be  made  on 
the  first  finding  of  sugar.  R.  Schmitz  also  emphasized  this  in  his  well 
known  work. 

The  general  etiology,  the  pathogenesis  and  metabolic  changes 
which  have  aroused  interest  in  the  scientific  investigations  of  diabetes 
must  be  sought  for  in  treatises  which  consider  the  disease  in  adults  and 
also  in  certain  special  works  upon  the  subject.  Nothing  of  sufficient 
importance  could  be  said  in  a  few  words  and  this  is  not  the  proper  place 
for  a  lengthy  consideration  of  the  subject.  Only  the  characteristic  con- 
ditions will  be  mentioned. 

Etiology. — Diabetes  in  childhood  attacks  boys  and  girls  with  appar- 
ently equal  frequency.  Some  statistics  indicate  a  slight  preponderance 
of  the  female  sex  while  among  adults  almost  twice  as  many  men  as 
women  are  affected.  Heredity  seems  to  me  to  be  much  less  marked  than 
in  adults,  although  there  are  instances  where  it  plays  an  important 
part.  I  have  recorded  the  medical  history  of  a  family  in  which  there 
was  a  mild  case  of  diabetes  in  the  first  generation,  three  female  members 
of  the  second  generation  developed  the  disease  at  middle  life  and  two 
children  of  the  tliird  generation  died  from  severe  and  rapid  types  of 
the  disease. 

It  is  a  very  common  experience  that  cases  of  diabetes  among 
children  do  not  occur  isolated  in  a  family.  Several  members  are  usually 
affected,  not  at  the  same  time  but  one  after  the  other  when  they  reach 
a  definite  age.  This  was  true  in  more  than  one  third  of  the  fifty  cases 
of  diabetes  in  childhood  treated  by  me.  If  the  family  history  is  closely 
investigated  it  is  often  found  that  the  parents  are  blood-relatives  or  that 
in  a  previous  generation  the  marriage  of  relatives  occurred.  This 
confirms  the  opinion  based  on  other  grounds  that  diabetes  in  cliildren 
as  well  as  in  many  of  the  cases  in  adult  fife  must  be  regarded  as  an  en- 
dogenous degenerative  disease.  The  well-recognized  frequency  of  dia- 
betes in  the  Jewish  race  probably  depends  upon  the  insufficient  admix- 
ture of  different  strains  of  blood.  Tlie  Jewish  race  certainly  shows  a 
marked  tendency  to  diabetes  in  childhood  but  not  to  my  mind  in  the 
same  degree  as  among  the  adults.  Besides  hereditary  influences,  trauma 
(concussion  of  the  brain)  is  often  mentioned  as  a  cause  of  diabetes  in 
children, — whether  correctly  seems  to  me  certainly  more  doubtful  than 
in  adults. 

Tills  is  to  be  especially  emphasized  as  we  must  regard  progressive 
diabetes  in  childhood  as  pancreatogenous  with  at  least  the  same 
certainty  as  orcUnary  diabetes.  The  examination  of  the  pancreas 
macroscopically  and  microscopically  reveals  so  few  anatomical  changes 
that  in  many  of  the  older  autopsy  records  it  was  not  deemed  necessary 
to  mention  its  condition.     In  the  last  two  decades  attention  has  been 


DIABETES  MELLITUS  S^l 

directed  to  the  pancreas.  The  small  size  and  relaxed  condition  of  that 
organ  has  been  given  as  a  frequent  finding.  I  myself  have  noted  the 
latter  condition,  although  no  changes  were  discovered  in  the  islands  of 
Langerhans.  It  is  of  interest  and  deserves  further  observation  that 
some  of  the  children  treated  by  me  for  diabetes  had  syphihtic  fathers 
and  that  that  disease  was  not  completely  cured  at  the  time  of  the  pro- 
creation of  the  child.  In  such  cases  it  is  possible  that  there  might  be  a 
functional  weakness  of  the  pancreas  due  to  the  syphilitic  virus.  I  have 
thought  of  this  only  recently  and  cannot  fortify  it  with  any  great  amount 
of  clinical  material. 

Symptoms. — Course  of  Disease. — From  the  \\Titings  upon  diabetes 
in  cliildhood,  the  impression  is  frequently  gained  that  the  onset  of  the 
disease  is  usually  quite  sudden  and  that  the  disease  begins  at  once  as  a 
severe  type  of  glycosuria.  My  experience  does  not  agree  with  this  opinion 
since  in  the  majority  of  my  httle  patients  there  were  periods  of  months 
or  even  years  during  which  the  glycosuria  was  of  a  mild  type  and  imme- 
diately modified  by  the  exclusion  or  even  moderate  hmitation  of  carbo- 
hydrates. Tliis  knowledge  has  been  gained  by  the  fact  that  the  urine 
of  small  children  is  tested  for  sugar  more  frequently  than  formerly. 
Cases  which  are  regarded  as  severe  directly  after  the  detection  of  the 
disease,  have  probably  not  been  observed  in  the  early  stages.  The 
passage  from  a  mild  form  to  a  severe  type  is  therefore  apparently  much 
more  rapid  in  children  than  in  adults.  So  long  as  the  cUsease  is  mild,  there 
is  little  evidence  of  illness.  The  thirst  may  betray  it  or  the  flecks  of 
sugar  on  the  underclothes  may  attract  the  mother's  attention.  Com- 
pUcations  such  as  disorders  of  the  skin,  diseases  of  the  eye,  neuralgias, 
etc.,  wliich  in  adults  so  often  give  the  first  diagnostic  liint  are  practi- 
cally unknown  in  the  diabetes  of  childhood.  When  the  diet  is  regu- 
lated, the  thirst  disappears  and  the  children  develop  satisfactorily  in 
their  physical  and  mental  growth. 

After  months  or  years  the  tolerance  for  carbohydrates  fails.  Tliis 
is  often  induced  by  some  foolish  lapse  in  diet  or  oftener  by  an  inter- 
current febrile  disease  (tonsilUtis,  diphtheria,  pneumonia,  influenza,  etc.), 
which  so  often  even  in  the  diabetes  of  adults  produces  a  rapidly  incurable 
change.  Even  when  such  causes  are  absent  the  lessened  tolerance  is 
only  postponed,  not  removed  and  the  diminution  quickly  changes  into 
complete  loss.  A  period  of  a  few  months,  often  but  several  weeks  may 
elapse  between  a  tolerance  for  80-100  grams  of  bread  and  the  complete 
development  of  a  severe  type  of  glycosuria,  no  longer  modified  by  the 
withdrawal  of  carbohydrates.  As  soon  as  the  loss  of  tolerance  appears, 
the  vivacity  of  the  child  with  the  physical  and  mental  acti^aty  disap- 
pears. They  do  not  want  to  play  with  other  children,  become  easih' 
exhausted,  complain  of  pains  in  the  joints  after  every  exertion  and 
rapidly  emaciate.     A  carefully  selected  dietary  and  good  nursing  may 


222  THE   DISEASES   OF   CHILDREN 

possibly  coax  back  the  old  \'igor  but  it  is  never  more  than  a  coaxing. 

In  the  meanwhile  thirst,  wliich  had  for  a  time  been  in  abeyance, 
reappears  and  the  quantity  of  urine  increases  two  to  four  times  the  nor- 
mal. The  urine  contains  large  amounts  of  acetone,  diacetic  and  ox}-- 
butjTic  acids  and  ammonia  and  the  breatli  has  the  odor  of  acetone 
exactly  the  same  as  in  adults.  The  fully  developed  picture  of  diabetic 
autointoxication  (diabetic  acidosis)  is  now  exident.  The  urine  is  rarely 
free  from  albumin  although  the  quantity  is  small.  Under  the  micro- 
scope the  so-called  coma  casts  are  seen  soon  after  the  first  appearance 
of  the  iron  chloride  reaction  and  their  number  markedly  increases  toward 
the  end  of  hfe.  I  found  the  largest  amounts  of  pathological  acid,  meta- 
bohc  products  among  cliildren  under  seven  years  of  age  in  a  boy  of 
four  years,  4.2  grams  of  acetone,  38. 5  grams  of  oxybutyric  acid  and  the 
urine  contained  4.5  grams  of  ammonia  in  an  excretion  of  10.2  grams  of 
urea.  In  tliis  patient  I  determined  the  finding,  repeated  in  other  cases, 
that  the  uric  acid  was  abnormally  abundant  on  an  absolutely  purin  free 
diet  (eggs,  vegetables,  butter,  cream,  oatmeal):  0.6-0.87  grams  per  day 
while  the  nitrogen  excretion  balanced  the  intake.  This  indicated  an 
enormous  nuclear  destruction  as  the  nuclein  is  the  progenitor  of  uric 
acid  and  the  other  purin  boches. 

The  termination  of  diabetes  in  cliildhood,  when  an  intercurrent 
infectious  disease  does  not  complicate  it,  is  without  exception  death  by 
coma.  Its  approach  is  usually  made  manifest  by  gastric  disorders  such 
as  loss  of  appetite,  nausea,  vomiting,  pain  in  stomach,  spontaneous  or 
on  pressure.  Increasing  nervous  irritabihty  alternating  with  rapid 
relaxation,  sleeplessness,  and  great  muscular  weakness  are  further 
symptoms.  They  often  continue  for  weeks  although  commonly  the  dis- 
ease runs  a  rapid  course.  No  mention  need  be  made  of  the  compHcating 
organic  diseases  occurring  with  diabetes  and  so  common  in  the  adult 
type  since  they  are  only  suggested.  Some  cases  have  been  found  asso- 
ciated with  an  unknown  functional  change  in  the  pancreas  and  disorder 
of  the  intestinal  secretion  (calculus  formation  in  the  duct  of  Wirsung 
with  resulting  cyst  and  destruction  of  gland).  Severe  thsorders  in  the 
digestion  especially  steatorrhnea  and  azotorrhtra  follow. 

The  prognosis  is  almost  without  exception  unfavorable  if  the  cUag- 
nosis  of  a  true  diabetes  is  certain.  As  already  stated  transitory  glyco- 
surias occur  especially  among  children  and  these  completely  recover.  R. 
Schmitz  and  G.  Klemperer  have  mentioned  such  cases  and  I  have  seen 
.several.  Such  diabetic  glycosurias  dependent  upon  tran.sitory  disorders 
of  the  pancreas  must  entire!}'  disappear  within  a  few  weeks,  if  the  seri- 
ousness of  the  prognosis  is  to  be  disregarded.  There  are  also  patients 
through  whose  entire  hfe  from  early  childhood  to  advanced  age,  a  definite 
intolerance  exists  to  large  quantities  of  carbohytlrates  which  is  not  pro- 
gressive in  character.     These  are  benign  cases. 


DIABETES  MELLITUS  223 

I  know  a  family,  the  father  of  whom  showed  from  his  sixth  year 
glycosuria  as  soon  as  the  quantity  of  carbohydrates  exceeded  200  grams. 
This  idiosyncrasy  has  continued  without  change  up  to  the  present  time. 
In  the  daughter,  glycosuria  has  never  been  detected.  One  of  two  sons 
had  even  in  liis  fourth  year  the  same  idiosyncrasy  as  liis  father  and 
continues  to  manifest  it  although  now  over  thirty  years  of  age.  Father 
and  son,  with  this  exception,  are  perfectly  healthy.  The  process 
has  possibly  been  influenced  by  the  fact  that  in  both  since  the  day 
of  discovery  of  tliis  condition,  there  has  been  a  rigid  reduction  in 
carbohydrates. 

With  few  exceptions  the  statement  is  true  that  true  diabetes  in 
childhood  knows  no  cure,  no  matter  how  mild  it  may  appear  in  the 
beginning  nor  how  gradual  its  development  in  the  first  months  or  even 
years. 

Treatment. — Treatment  has  no  effect  in  preventing  this  sad  result 
but  may  inliueuce  the  duration  of  the  disease.  This  has  usually  been 
given  in  the  wTitings  of  others  as  one  to  one  and  a  half  years.  The  aver- 
age duration  of  my  cases,  which  were  detected  after  their  development 
into  a  severe  type,  was  not  much  higher;  one  and  a  half  to  two  years. 
Patients  who  came  under  observation  in  the  stage  of  mild  glycosuria 
lived  three  to  six  years.  Only  those  are  considered  in  whom  the  disease 
developed  before  the  seventh  year  of  life. 

In  spite  of  the  hopeless  prognosis,  it  is  oui'  duty  to  prolong  life  as 
much  as  possible.    The  treatment  will  vary  with  the  stage  of  the  disease. 

As  soon  as  the  tolerance  for  carbohydrates  has  been  reduced  to  noth- 
ing, or  has  gone  beyond  that,  strict  dietary  rules  need  no  longer  be 
considered.  Their  value  no  longer  equals  the  distress  which  the  com- 
plete prohibition  of  the  carbohydrates  or  the  hmitation  of  the  proteid 
diet  gives  to  the  child.  Carbohydrates,  with  the  exception  of  sugar,  are 
permitted  and  it  is  a  matter  of  indifference  whether  emphasis  is  placed 
upon  milk  or  upon  cereals.  Experience  however  will  teach  that  the 
carbohydrates  of  oatmeal  are  by  far  best  assimilated  in  the  diabetes  of 
cliildren.  It  has  been  possible  for  me  several  times  to  reproduce  for  a 
time  a  marked  tolerance  for  carbohydrates  by  an  oatmeal  cure.  Lang- 
stein  also  noted  favorable  results  from  its  use  in  children.  In  the  oat- 
meal cure  children  receive  notlaing  except  a  gruel  made  of  150  grams 
(5  oz.)  of  oatmeal,  150-200  grams  (5  to  6J  oz.)  of  butter,  60-70  grams 
(2-2^  oz.)  of  Roborat  or  4-5  eggs  as  a  daily  allowance  and  in  addition 
some  wine.  Tliis  diet  is  continued  1-2  weeks  and  then  gradually  replaced 
by  other  food.  The  result  is  often  marvellous  in  increasing  the  toler- 
ance, which  unfortunately  does  not  continue.  Alkahes  are  the  only 
drugs  to  be  considered  unless  there  are  distinct  indications  for  other 
medication;  10-15  grams  (5iiss-5iv)  of  bicarbonate  of  soda  are  adminis- 
tered daily  to  neutralize  the  acid  products  of  metabohsm  and  to  prevent 


224  THE    DISEASES   OF    CHILDREN 

acid  intoxication.  When  there  is  a  marked  tolerance  for  carbohydrates 
at  least  to  the  extent  of  about  40-50  grams  (1^-2  oz.)  mth  a  diet  other- 
wise strictly  free  from  carbohydrates  (meats,  eggs,  green  vegetables, 
fats),  tliis  favorable  condition  with  complete  physical  and  mental  Adgor 
of  the  child  may  be  prolonged  for  a  considerable  period.  To  accomplish 
this,  an  exact  knowledge  of  the  limits  of  tolerance  is  necessary.  The 
quantity  of  the  carbohydrates  allowed  must  then  be  kept  within  these 
limits  and  tliis  should  be  alternated  from  time  to  time  for  several  days 
with  a  strict  carbohydrate-free  diet.  It  is  unfortunately  impossible  to 
arrange  distinct  schemata  for  such  a  diet  since  the  excessive  capricious- 
ness  of  the  taste  in  childhood  makes  each  patient  an  object  of  special 
study.    Schematic  regulations  are  from  their  nature  worthless. 

It  is  a  difficult  matter  for  the  child  and  still  more  for  the  relatives 
who  are  responsible  to  continuously  administer  carbohydrates  and  pro- 
teids  below  the  Umit  which  produces  glycosuria  and  at  the  same  time  to 
satisfy  the  demands  of  the  infantile  digestion  and  the  taste  of  the  cliild. 
But  it  must  be  done  if  the  child  is  to  be  brought  through.  This  attempt 
has  so  rarely  been  scrupulously  made,  that  httle  can  be  said  of  the  gen- 
eral results  of  such  treatment.  It  would  be  of  great  importance  in  many 
cases  to  carry  through  the  chetetic  treatment  of  the  child  in  a  sanitarium, 
with  the  mother  or  another  member  of  the  family.  It  might  then  be 
feasible  to  restrain  as  long  as  possible  the  advances  of  the  morbid  pro- 
cesses and  thereby  to  give  opportunity  to  the  organism  to  overcome  the 
disease  in  case  it  is  not  of  a  hopelessly  malignant  nature. 

It  is  naturally  senseless  to  recommend  for  diabetic  cliildren  thQ 
cure  at  Carlsbad,  Neuenahr  or  Vichy.  Of  drugs,  none  can  be  recom- 
mended. Many  home  remedies  are  praised  for  diabetes  in  infants  as 
well  as  for  that  disease  in  adults  but  such  praise  is  almost  criminal. 
Alkalies  should  not  be  administered  before  the  condition  of  the  urine 
(acetone,  acetic  acid  and  oxybutyric  acid)  indicates  the  proximity  of 
an  acid  intoxication.  It  is  not  wise  to  begin  earlier  as  children  do  not 
bear  alkalies  well  for  a  long  time  and  frequently  digestive  disturbances 
result  from  their  use. 

When  diabetic  coma  occurs  the  attempt  can  be  made  to  overcome 
it  by  intravenous  infusions  of  a  3  per  cent,  solution  of  soda.  It  has  been 
possible  in  some  cases  to  get  a  good  result,  the  fatal  termination  how- 
ever is  only  postponed. 


DIABETES  INSIPIDUS 

BY 

Professor  C.  von  NOORDEN,  of  Vienna 

translated  by 
Dr.  ANDREW  MACFARLANE,  Albany,  N.  Y. 


Diabetes  Insipidus  is  a  disease  characterized  by  the  secretion  of  an 
abnormally  large  quantity  of  urine  which  contains  no  sugar  and  shows 
no  affection  of  the  kidneys.  The  concentration  of  the  urine  is  relatively 
less  than  the  quantity;  the  specific  gravity  often  registers  1.005  and 
lower  and  the  color  is  abnormally  light.  The  great  loss  of  water  through 
the  kidneys  increases  the  thirst  (polydipsia)  and  diminishes  the  excre- 
tion of  water  by  the  skin,  which  as  a  rule  is  dry  and  roughened. 

The  disease  is  rare — rarer  than  diabetes  meUitus  although  rela- 
tively more  frequent  in  childhood.  Ten  to  fifteen  per  cent,  of  the  total 
number  affected  occur  in  the  first  decade  but  the  majority  of  these  in 
the  second  half  of  tliis  decade. 

Etiology. — A  constant  pathologic-anatomical  basis  for  diabetes 
insipidus  has  not  been  discovered.  Diseases  of  the  cerebellum  and 
especially  of  the  medulla  may  show  CAadence  of  diabetes  insipidus 
but  it  is  doubtful  if  these  cases  are  identical  in  their  pathogenesis  with 
those  in  which  no  anatomical  lesion  of  the  brain  whatever  is  found. 
Cerebral  concussion  also  plays  an  undoubted  role.  Polyuria  often  de- 
velops towards  the  end  of  an  acute  infectious  disease,  increases  to  a  dis- 
tressing degree,  continues  many  weeks  beyond  the  primary  disorder 
and  then  gradually  returns  to  normal.  Tliis  condition  should  not  be 
classified  as  true  diabetes  insipidus  but  at  most  as  a  symptomatic  form 
of  the  disease.     The  etiology  and  pathogenesis  are  generally  unknown. 

Symptomatology. — Diabetes  insipidus,  if  it  is  not  a  postinfec- 
tious polyuria,  is  practically  always  a  serious  disease  in  childhood 
whether  it  develops  in  the  train  of  a  cerebral  disease  or  appears  spon- 
taneously. Cliildren  suffer  much  from  the  distressing  thirst,  take  no 
pleasure  in  their  play  or  work,  become  irritable  and  quickly  exhausted. 
A  gradual  emaciation  almost  always  occurs,  due  to  the  difficulty  of 
administering  sufficient  nourishment  because  of  the  large  quantity  of 
fluids  which  they  drink.  Considerable  loss  of  heat  results  from  rais- 
ing this  large  quantity  of  fluid,  usually  drunk  cold,  to  the  temperature 
of  the  body.  I  estimated  in  one  patient,  a  boy  ten  3^ears  of  age,  that  tliis 
loss  of  heat  increased  the  calorimctric  needs  of  the  body  about  13  per 
cent,  more  than  normal.  These  children  usually  are  for  their  age  mark- 
edly deficient  in  growth  and  especially  in  the  development  of  muscle 

11—15  225 


226  THE   DISEASES   OF   CHILDREN 

and  bone.  No  other  change  in  metabolism  has  yet  been  discovered. 
Although  the  secretion  of  urine  may  reach  three  to  four  quarts  in  moder- 
ately severe  cases,  and  seven  to  eight  quarts  and  more  in  severe  cases 
even  in  children,  the  constituents  of  the  urine  (urea,  uric  acid,  mineral 
salts)  are  present  in  normal  amount.  The  urine  often  but  not  always 
contains  inosit,  the  significance  of  which  however  is  still  in  doubt. 

Other  symptoms  and  retrograde  changes  are  lessened  perspiration, 
often  some  reduction  in  the  temperature  of  the  body,  marked  concen- 
tration of  the  blood  serum,  trophic  changes  in  the  nails,  defective  growth 
of  hair,  rarely  forms  of  neuritis,  especially  optic  neuritis. 

The  diagnosis  is  easily  made  from  the  symptoms.  It  is  only 
necessary  to  decide  whether  it  is  a  true  diabetes  insipidus  or  a  symp- 
tomatic polyuria. 

The  prognosis  and  course  cannot  be  predicted  with  certainty. 
It  is  dependent  in  diseases  of  the  brain  much  more  upon  the  primary 
condition  than  upon  the  diabetes  insipidus.  ^Yhen  the  disease  occurs 
spontaneously  and  becomes  fully  developed,  it  usually  goes  on  to  a 
fatal  termination  by  gradual  exhaustion  or  by  some  intercurrent  dis- 
ease (tuberculosis)  for  which  it  furnishes  the  soil.  The  prognosis 
however  is  not  nearly  as  serious  as  in  diabetes  melUtus  since  complete 
recoveries  and  in  other  cases  improvements  have  occurred.  A  well- 
defined  polyuria  and  polydipsia  may  continue  through  life  and  be 
regarded  as  an  inconvenience  rather  than  a  disease. 

Treatment  is  not  entirely  without  effect.  Systematic,  careful 
and  graded  restriction  of  fluids  may  produce  beneficial  and  permanent 
results.  I  have  seen  several  of  these  favorable  cases  among  children. 
Hospital  treatment  is  often  more  effective  than  that  at  home.  Ex- 
clusive diets,  as  meat,  millc  or  vegetable,  have  been  strongly  recom- 
mended but  cannot  be  enforced.  The  care  and  nourishment  should  be 
directed  to  strengthening  the  body  as  much  as  possible.  The  question 
has  recently  arisen  if  it  would  not  be  possible  to  reduce  the  exchange  of 
fluids  in  the  body  by  a  salt  free  diet  and  thus  induce  a  gradual  return 
to  normal  conditions.  This  deserves  further  investigation.  Recently 
a  child  suft'ering  with  diabetes  insipidus  recovered  under  this  treatment 
in  my  hospital  service.  Everytliing  which  stimulates  the  peripheral 
circulation  is  to  be  recommended.  A  constant  out-of-door  life  has  often 
a  marlved  effect  upon  the  polyuria  and  polydipsia.  Favorable  results 
have  been  reported  from  the  use  of  the  sulphur  baths  at  Kreuznach 
and  Nauheim  and  recently  air  and  sun  baths  have  been  extolled. 

Almost  every  drug  has  been  tried  and  especially  opium,  bella- 
donna, strychnine,  ergotin,  pilocarpin,  antipyrin  and  the  sahcylates, 
recently  adrenaUn.  On  account  of  the  great  uncertainty  in  their  action 
only  the  temporary  use  of  such  powerful  drugs  has  seemed  justified  in 
children. 


SCROFULA 

BY 

Dr.  B.  SALGE,  ok  Dresden 

translated  by 
Dr.  GODFREY  R.  PISEK,  New  York.  N.  Y. 

ScROFUL.\.  is  a  disease  of  childhood,  and  of  puberty,  occurring  some- 
what more  often  in  the  female  than  in  the  male  sex. 

The  name  "Scrofula"  is  derived  from  Skropho,  I  root:  and  .signi- 
fies a  young  swine.  The  basis  for  this  term  was  very  likely  suggested 
by  the  striking  disfigurement  of  the  face  and  neck  occurring  in  typical 
cases,  and  which  indeed  renunds  us  of  the  appearance  of  a  pig. 

Historical. — At  the  beginning  of  the  last  century,  the  name  scrof- 
ula was  ver}-  often  used  synonymously  with  tuberculosis.  Lannec 
.considered  scrofula  nothing  more  than  a  tuberculous  disease  of  the 
glands  and  in  this  conception  had  many  followers. 

Virchow  combated  this  opinion.  For  him,  the  tubercle  alone  is 
characteristic  of  tuberculosis,  not  the  caseous  degeneration  upon  which 
Laennec  laid  special  weight,  since  this  may  occur  also  in  other  conditions, 
as  cancer,  etc. 

The  scrofulous  affections,  according  to  Virchow  are  "irritative 
changes  in  the  tissues,  which  have,  partly,  a  hyperplastic,  and  partly 
an  inflammatory  character."  Scrofula  in  the  narrower  sense,  he  con- 
siders a  disease  of  the  lymphatic  nodes,  which  is  provoked  by  a  certain 
weakness,  or  imperfect  structure  of  the  respective  glandular  regions. 

The  discovery  of  the  tubercle  bacillus  by  Koch,  led  to  the  positive 
proof,  that  joint  and  bone  disease,  lupus,  scrofuloderma  and  lichen 
scrofulosum  are  of  a  tuberculous  nature,  and  hence  belong  properly 
under  the  general  term,  tuberculosis,  and  must  be  distinguished  from 
scrofula.  That  tMs  distinction  is  not  always  made  is  to  be  explained 
by  the  fact  that  these  diseases  were  known  for  generations  by  the 
very  name  of  scrofula,  and  are  still  believed  to  be  scrofulous  changes 
by  the  laity. 

The  lymph-nodes,  considered  diagnostically  so  very  important  in 
scrofula,  showed  tuberculosis  not  only  in  the  nodes  presenting  histolog- 
ical changes,  but  in  addition  the  nodes  that  were  simply  enlarged  could 
be  proven  to  be  infected  with  tuberculosis  in  cut  sections,  and  especially 
by  the  serum  test.  Here  ought  to  be  added,  that  since  the  admirable 
researches  of  Bartel  and  of  the  Weichselbaum  School,  there  can  no 
longer  be  any  doubt  that  a  lymph-node  infected  by  tuberculosis  need 
not  always  show  specific  tuberculous  changes. 

227 


228  THE   DISEASES   OF   CHILDREN 

Even  though  it  is  quite  generally  admitted  that  the  above-men- 
tioned affections,  as  well  as  the  scrofulous  lymph-nodes  are  of  a  tuber- 
culous nature,  still,  the  affections  of  the  sldn  and  mucous  membranes 
are  not  always  so  considered.  These  latter  affections  do  not  as  a  rule, 
show  tubercle  bacilli,  and  for  that  reason,  as  many  authors  assert,  can- 
not be  classed  as  tuberculous.  Cornet  beUeves  that  the  changes  in  the 
skin  and  mucous  membranes  are  caused  not  only  bj'  the  tuliercle  bacilh, 
but  also  by  the  presence  of  pyogenic  bacteria,  such  as  the  staphylococci 
and  streptococci.  Still  it  is  not  quite  clear  why  these  infections  in  chil- 
dren lead  to  such  severe  and  stubborn  changes ;  as  do  not  take  place  in 
similar  bacterial  infections. 

We  will  now  discuss  the  indistinct  theory  called  Predisposition  to 
Scrofula;  an  expression  which  is  less  often  apphed  to  other  diseases,  or 
is  the  subject  of  so  much  contention  and  insufficient  explanation.  Even 
the  views  of  Virchow  as  referred  to  above,  contained  such  ideas  on  this 
subject,  without  telhng  us  of  what  the  assumed  weakness  or  insufficient 
functions  of  the  respective  lymph-nodes  consisted. 

Cornet  in  his  well-known  work  on  scrofula,  devoted  an  entire  chap- 
ter to  predisposition  to  scrofula,  and  explains  its  cause  in  the  greater 
permeabifity  of  the  skin  and  mucous  membranes,  to  bacteria,  and  the 
greater  number  of  lymph-passages.  He  tries  to  prove  that  this  per- 
meabifity in  the  organism  of  the  child,  as  compared  with  that  of  the 
adult,  is  naturally  greater,  and  is  enhanced  in  certain  incfividuals,  fur- 
nishing thus  the  predisposition  to  scrofula.  He  calls  tliis  condition 
Increased  Infantifism  or  Embryonafism.  Unfortunately  however,  ana- 
tomical findings  are  lacking  to  verify  this  embryonal  condition  in 
scrofulous  individuals.  While  Cornet  completely  rejects  the  theory  of 
a  precfisposition  affecting  the  entire  human  system,  Czerny  endeavored 
to  find  this  predisposition  in  the  chemical  composition  of  the  organism, 
asserting  that  for  a  predisposition  to  infectious  diseases,  we  must  assume 
an  alteration  of  the  chemical  compositions  of  the  body.  But  no  chem- 
ical examinations  to  substantiate  this  theory  have  as  yet  been  made. 
It  may  be  here  briefly  mentioned  that  Czerny's  idea  differs  greatly  from 
the  generally  accepted  conceptions  of  scrofula.  Only  that  which  can 
be  proven  histologically  or  bacteriologically  as  tuberculous  is  actually 
tuberculosis  and  is  to  be  distinguished  from  scrofula.  Czerny's  defini- 
tion of  the  term  scrofula  did  not  lead  to  a  clearer  understanding,  nor  to 
a  better  classification  of  the  affection ;  on  the  contrary,  an  old  name, 
which,  heretofore,  was  apphed  to  other  conditions,  was  made  to  serve  as 
a  name  for  a  sympton-complex.  Recently  however,  Czerny  has  re- 
nounced the  name  scrofula,  for  liis  special  series  of  symptoms  and 
classified  the  respective  signs  in  accordance  with  liis  new  theory,  under 
the  name  "exudative  diathesis,"  which  will  be  spoken  of  in  another 
part  of  this  book.    It  must  be  mentioned  here,  that  a  part  of  the  patho- 


SCROFULA  229 

logical  phenomena,  arranged  by  Czerny  under  the  name  "exudative 
diathesis,"  are  still  embodied  under  the  old  name  scrofula.  Others  how- 
ever, may  be  beheved  to  be  conditions,  which,  according  to  Heubner, 
could  be  called  Lymphatismus.  Czerny's  ingenious  interpretations 
show  very  clearly  our  perfect  ignorance  of  what  the  real  nature  of  tliis 
diathesis  actually  is.  Classify  these  conditions  as  one  will,  future 
chemical  examinations  may  show  better  criteria ;  until  then  we  must 
be  content  with  such  terms  as:  "peculiar  tendency,"  "diathesis,"  etc. 
That  hereditary  influences  may  play  an  important  part  in  the  develop- 
ment of  "scrofulous  tendencies,"  is  perhaps  universally  acknowledged. 
And  indeed,  it  \\ill  be  noticed,  that  those  children  are  mostly  attacked 
by  scrofula,  whose  parents  had  the  same  disease  or  tuberculosis  in  their 
youth,  and  the  appellation,  "scrofulous  families,"  as  used  by  the  people 
at  large  must  be  acknowledged  as  justified.  As  has  been  noted  laymen 
even  now  consider  tuberculosis  of  the  joints,  bones  and  skin  as  mani- 
festations of  scrofula. 

Owing  to  the  extraordinary  influence  of  heredity,  Soltmann  made 
an  attempt  to  explain  the  predisposition  to  scrofula  and  the  tendency 
to  tuberculosis  in  such  in^^i^^duals,  and  it  is  worth  while  to  mention  it. 
Before  I  consider  this  theory,  I  wish  to  state  that  "heredity  in  tuber- 
culosis" will  be  treated  in  the  chapter  on  tuberculosis.  Soltmann 
assumes  that  not  the  actual  bacilli  enter  into  the  foetus,  but  rather  the 
toxins  produced  by  the  tubercle  bacilH,  and  these  toxins,  passing  through 
the  placenta,  poison  the  developing  organism.  The  result  of  such  poison- 
ing it  is  believed,  is  the  cause  of  a  scrofulous  constitution  \\'ith  a  ten- 
dency toward  acquiring  tuberculosis.  The  chathesis  would  have  to  be  at 
once  diagnosticated  as  a  sign  of  tuberculosis,  in  the  sense  of  a  toxic 
infectious  disease,  and  the  tendency  for  such  individuals  to  take  tuber- 
culosis, would  be  nothing  more  than  an  over-sensitiveness  of  the  already 
poisoned  organism  to  the  tuberculous  \drus — a  theory  well  shown  in 
this  disease  from  our  knowledge  of  tubercuhn. 

Of  course  all  this  is  only  hypothetical,  and  as  yet  entirely  unproven, 
inasmuch  as  it  presupposes  the  circulation  of  tuberculous  toxines  in  the 
body  of  the  pregnant  mother.  This  is  hardly  a  plausible  assumption. 
But  suppose  we  were  inclined  to  reject  such  a  direct  influence  as  hered- 
ity, we  must  still  admit  that  children  of  tuberculous  parents  are  very 
frequently  subjected  to  the  possibihty  of  infection  from  tubercle  bacilli 
and  for  that  reason  very  frequently  become  ill;  even  this  opinion  is  not 
to  be  accepted  without  reserve  for  not  all  parents  supposedly  afflicted 
with  tuberculosis,  and  whose  children  fall  ill  -nith  scrofula,  have  tuber- 
culosis in  such  a  form  as  to  infect  their  surroundings  with  tubercle  bacilU. 

It  must  be  remembered,  that  in  recent  times,  the  view  expressed 
by  von  Behring  on  the  subject  of  infection  by  tubercle  bacilli  in  nurs- 
lings is  receiving  greater  confirmation,  but  it  does  not  by  any  means 


230  THE   DISEASES   OF   CHILDREN 

follow  that  a  true  case  of  tuberculosis  results,  instead  it  often  happens 
that  the  infection  remains  latent.  Vnder  such  circumstances  the  devel- 
opment of  a  case  of  scrofula  is  a  sign  that  an  infection  with  tuberculous 
virus  has  taken  place. 

All  these  speculative  arguments  make  it  clear  that  at  present 
there  is  no  explanation  for  the  hereditary  influence  that  undoubtedly 
exists;  in  other  words  we  must  accept  the  theory  of  an  hereditary  predis- 
position although  nobody  can  tell  in  what  it  consists. 

The  question  now  arises,  What  are  the  relations  of  the  individual 
symptoms  of  scrofula  to  tuberculosis?  It  has  been  said  that  so  far  as 
the  glands  are  concerned,  their  tuberculous  nature  is  generally  con- 
ceded, but  opinions  differ  greatly  as  to  the  conditions  found  in  aUied 
inflammations  of  the  skin  and  mucous  memljranes.  It  is  true  that  in 
such  cases  tubercle  bacilli  are  not  found,  but  a  great  variety  of  other 
bacteria,  as  staphylococci,  etc.  Such  bacteria  when  found  on  the  sur- 
face of  the  skin  and  mucous  membranes,  prove  nothing  at  all.  At  best, 
they  may  be  of  secondary  importance,  inasnmch  as  the  inflamed  and 
diseased  surface  facihtates  or  favors  their  penetration.  That  the 
staphylococci,  etc.,  may  cause  suppuration  and  abscesses,  is  readily 
understood;  but  it  is  not  by  any  means  exadent  that  the  symptoms  diag- 
nosticated as  scrofula  are  caused  by  these  bacteria.  On  the  contrary,  this 
is  even  highly  improbable,  for  true  infection  with  pus  cocci  in  a  young 
child  shows  different  characteristics.  Compare  for  instance  the  course 
of  a  scrofulous  disease  of  the  eyes,  as  described  liclow,  with  the  suppura- 
tive condition.  In  the  latter  we  do  not  find  a  circumscribed  focus,  the 
vesicular  eruption,  but  a  diffuse  catarrh,  accompanied  by  an  abun- 
dance of  secretion.  The  prototype  of  this  condition  is  the  gonorrhoeal 
inflammation,  wliich  never,  even  in  most  stubborn  cases,  assumes  any- 
thing like  the  characteristics  of  a  scrofulous  eye.  The  same  may  be  said 
of  other  pyogenic  agents,  the  staphylococci,  for  example.  Inflamma- 
tions present  a  good  opportunity  to  study  such  infections  of  the  eye, 
manifesting  themselves  by  a  deficient  winking  and  closure  of  the  eye- 
lids in  severe  intestinal  affections.  In  case  we  succeed  in  keeping  such 
a  child  aUve,  the  ocular  suppuration  may  extend  over  the  entire  period 
of  convalescence,  which  may  last  for  weeks,  but  it  never  assumes  such 
characteristics  as  might  cause  it  to  be  confounded  with  scrofulous 
disease  of  the  eyes. 

It  is  the  same  with  affections  of  the  skin  and  mucous  membranes. 
Infections  of  the  skin  with  staphylococci  lead  to  the  formation  of  fur- 
uncles, to  abscesses  and  to  inflammation  and  suppuration  of  the  glands 
involved,  but  they  do  not  produce  that  pecuharly  stubborn  scrofulous 
catarrh,  tending  toward  a  state  of  hyperplasia  and  ulceration.  We  may 
observe  in  a  poorly  nourished  child  afflicted  with  chronic  intertrigo  a 
great   many  skin   affections  in  conjunction   with  suppuration,   but   no 


SCROFULA  231 

changes  characteristic  of  scrofula.  Hence  it  is  quite  an  arbitrary  as- 
sumption to  confound  the  scrofulous  diseases  of  the  skin  and  mucous 
membranes  with  infections  by  bacteria  and  especially  the  pus  cocci. 

To  explain  this  we  must  again  resort  to  predisposition,  a  peculiar 
but  unproven  anatomical  condition.  But  even  granting  such  a  pecu- 
har  anatomical  condition,  and  such  a  singular  disposition  on  the  part  of 
the  tissues,  there  still  remains  to  be  explained  the  theory  why  it  is  that 
in  these  inchviduals  mth  such  peculiar  disposition,  bacteria  of  abso- 
lutely different  biological  effectiveness,  as  tubercle  bacilli  and  pus  cocci, 
produce  at  once  the  same  anatomical  changes,  which  they  otherwise 
never  do  as  a  rule. 

It  must  not  be  inferred  that  tubercle  bacilli  are  not  present,  because 
pus  is  produced,  or  because  they  cannot  be  found.  Heubner  in  his  text 
book,  teaches  as  follows:  The  peripheral  appearance  of  scrofula  is  imme- 
diately succeeded  b}-  an  infection  of  the  glands  with  tubercle  bacilli,  and 
it  is  inconceivable,  why  these  catarrhs  should  onl}'  furnish  the  oppor- 
tunity for  the  entrance  of  tubercle  bacilU,  nor  is  it  quite  clear,  why  this 
occurs  just  here,  and  not  in  other  inflammatory  conditions  of  the  same 
organs.  Moreover,  it  must  be  emphasized  that  nearly  all  scrofulous 
children  and  even  those  who  do  not  yet  show  any  glandular  swelHng, 
react  with  tuberculin  and  also  possess  in  their  serum  the  power  to  agglu- 
tinate tubercle  bacilli. 

Finally,  we  regularly  find  as  Heubner  says,  in  necropsies  of  such 
cases  of  purely  scrofulous  catarrhs,  tuberculosis  of  the  bronchial  glands. 
It  is  more  sensible  therefore,  because  of  the  close  connection  existing 
between  scrofula  and  tuberculosis,  to  ascribe  even  these  peripheral 
affections  to  the  latter,  insead  of  to  something  else  wliich  nobody  has 
seen  nor  proven. 

It  has  also  been  observed  in  children,  with  pronounced  tubercu- 
losis, that  typical  scrofula  suddenly  appears.  It  has  been  mentioned 
above  that  latent  cases  of  tuberculosis,  can,  by  biological  investigations, 
be  detected  even  in  very  young  suckling  babies,  and  we  must  repeat 
the  assertion,  that  when  scrofula  later  develops  it  is  noticing  else  but 
evidence  of  the  already  existing  infection. 

Therefore,  it  appears  to  me  correct,  to  bcUeve  strongly  that  these 
scrofulous  catarrhs  also  are  tuberculous  in  nature. 

DISEASES  OF  THE  EYE 

Lymphatic  conjunctivitis  or  keratitis,  i.e.,  scrofulous  or  phlyc- 
tsenular  keratitis,  is  a  localized  disease,  distinguished  from  all  other 
inflammatory  affections  of  the  conjunctiva,  and  representing  rather  a 
diffused  catarrh  of  the  connective  tissue. 

The  disease  begins  with  the  appearance  of  a  conical  vesicle  in  the 
limbus,  together  with  fascicular,  and  radiating  vascular  injections.    At 


232  THE   DISEASES   OF   CHILDREN 

the  top  of  the  cone  an  abscess  forms,  which  spreads  until  it  reaches  the 
conjunctiva,  and  then  goes  on  to  a  cure.  Usually  there  are  a  number  of 
vesicles  wliich  may  develop  beyond  the  limbus,  and  even  on  the  cornea. 
These  httle  nodules  or  abscesses  often  heal  in  about  a  fortnight,  but 
relapses  are  very  frequent;  in  fact  almost  the  rule,  so  that  the  disease, 
first  attacking  one  eye,  then  the  other,  may  trouble  a  patient  for 
years.  The  vesicles  of  the  cornea  may  heal  without  opacity,  but,  on 
the  other  hand,  deep  abscesses  do  occur,  permeating  Bowman's  mem- 
brane, which  can  only  heal  with  permanent  opacity.  Furthermore  the 
ulceration  on  the  cornea  may  assume  a  serpigenous  character  and 
accompanied  by  vascular  adhesions  may  cover  larger  or  smaller  areas 
of  the  cornea.  Within  the  confines  of  this  vascular  adhesion  permanent 
opacity  regularly  occurs.  Finally  instead  of  a  locahzed  affection,  a 
diffuse  neoplasm,  which  is  known  as  "  Pannus  scrofulosus  corne£e"  may 
develop.  This  neoplasm  is  generally  thin  and  capable  of  disappearing. 
Photophobia  is  the  subjective  symptom  of  paramount  importance. 
It  is  nearly  always  present  and  severe  in  form;  less  frequently  it  is  of  a 
mild  character.  The  children  retire  into  dark  corners,  lie  down  on  their 
faces,  press  their  heads  tightly  into  the  pillows  and  resist  most  energeti- 
cally any  attempt  to  open  their  eyes.  The  blepharospasm  may  reach 
so  severe  a  form  that  it  is  impossible  without  resorting  to  the  use  of  the 
speculum  to  see  the  condition  of  the  eye.  As  a  result  of  the  inflamma- 
tion there  is  a  profuse  flow  of  tears.  On  account  of  the  almost  constant 
moisture  on  the  lids,  blepharitis  in  an  intense  form  develops,  in  conse- 
quence of  which  an  irregular  position  of  the  ciha  as  well  as  eczema  of 
the  eyelids  may  be  observed.  This  latter  condition  may  in  permanent 
cases  become  so  intense,  that  it  may  lead  to  deformity  or  eversion  of 
the  lids,  e.g.  ectropion.  In  addition  to  this  in  long  standing  cases  as  a 
result  of  the  constant  irritation  of  the  tears,  a  diffuse  inflammation  of 
the  palpebral  conjunctiva  may  occur.  This  inflammation  as  such  has 
notliing  to  do  with  the  real  scrofulous  disease  of  the  eye.  In  summing 
up  all  of  the  evidence  we  come  to  the  conclusion  that  the  disease  is 
founded  upon  a  locahzed  affection  of  the  exterior  coverings  of  the  eye- 
ball. Its  stubbornness,  and  the  repeated  relapses,  indisputably  charac- 
terize its  scrofulous  nature.  Whether  the  chronic  diffuse  inflammation 
of  the  hds,  skin,  etc.,  which  arises  in  consequence,  is  caused  by  recur- 
rences of  the  scrofulous  disease  of  the  eyes  in  particular,  or  by  a  special 
vulnerabifity  of  the  tissues  in  chronic  scrofula  (habitus  scrofulosus) 
must  still  remain  a  mooted  question.  The  prognosis  of  the  ocular 
affections  is  in  general  a  favorable  one,  particularly,  if  the  severity  and 
long  duration  of  the  pathological  phenomena  are  taken  into  consider- 
ation. In  the  majority  of  cases  there  occur  only  slight  permanent 
injuries  to  the  power  of  vision.  Serious  disturbances  or  total  blindness 
are  very  rare. 


SCROFULA  233 

DISEASES   OF  THE   NOSE 

With  very  few  premonitory  symptoms  a  patient  is  attacked  by  a 
severe  and  remarkably  obstinate  chronic  cold  in  the  head.  There  is  a 
marked  swelling  of  the  submucosa.  A  limited  quantity  of  a  sticky 
mucoid  secretion  appears,  wliich  however  soon  dries  up  forming  a  crust 
and  soon  leads  to  intense  irritation  of  the  skin  and  to  excoriations  in  the 
nasal  orifices.  The  irritation  of  the  neighboring  skin,  particularly  of 
the  upper  lip,  causes  the  appearance  of  a  chronic  eczema,  which  at  first ' 
does  not  differ  from  any  other  eczema,  but  later  assumes  a  pecuHar 
character  of  its  own  on  account  of  its  great  obstinacy  and  the  chronic 
swelUng  of  the  affected  parts  which  appears  in  consequence  of  the 
infiltration  of  the  subcutaneous  connective  tissues.  The  pernicious 
effects  of  the  diseased  eyes,  described  above,  together  with  the  con- 
sequences arising  therefrom,  give  the  face  that  repulsive  appearance, 
peculiar  to  scrofula,  and  which  in  reahty  reminds  one  of  the  appear- 
ance of  a  pig  (see  Plate  11).  Of  course  the  secondary  changes  of  the 
skin  arising  from  the  ocular  disease  and  the  rhinitis  may  assume  differ- 
ent aspects.  The  eczema  is  apt  to  spread  over  the  entire  face  and  the 
hairy  scalp  as  well,  forming  thick  crusts.  Furthermore,  through  infec- 
tion of  the  eczematous  skin,  multiple  abscesses  and  ulcers  may  form 
which  stubbornly  resist  all  measures  used  for  their  cure.  Frequently 
the  eczema  extends  to  the  external  auditory  canal.  The  result  is  an 
intense  otitis  externa,  with  much  swelling  of  the  auditory  canal  and 
copious  formation  of  crusts  often  completely  obstructing  the  external 
auditory  canal.  This  condition  be  it  observed,  may  occur  without  an 
accompanying  otitis  media  ;  though  in  many  cases  this  does  exist  and 
through  its  secretions  there  may  result  eczema  of  the  auricle  and  the 
auditory  canal. 

OTITIS  MEDIA 

'V^Tiether  otitis  media  may  be  directly  accepted  as  a  symptom  of 
scrofula,  as  is  often  done,  or  not,  is  a  matter  of  opinion.  Otitis  media 
is  a  very  frequent  disease  of  cliildhood.  It  is  often  noticed  in  cliildren 
that  show  no  signs  whatever  of  scrofula.  The  condition  can  easily  be 
accounted  for  by  the  constant  possibihty  of  infection  from  rliinitis. 
But  at  the  same  time  it  cannot  be  denied  that  this  disease  of  the  ear 
proves  to  be  just  as  obstinate  as  other  affections  pronounced  as  scrofu- 
lous ;  nay  more,  it  clings  to  the  patient  even  after  the  disappearance  of 
scrofula  proper.  It  continues  into  the  later  period  of  childhood  and  not 
infrequently  results  in  very  serious  afflictions.  Chronic  otitis  media 
may  result  in  serious  affections  of  the  antrum,  and  of  the  mastoid  pro- 
cess. However  these  affections  do  not  differ  from  those  occuring  in 
non-scrofulous  individuals.  There  is  also  a  scrofulous  otitis,  having  its 
origin  in  an  infection  with  tubercle  bacilh.     Swelling  and  caseation  of 


234  THE    DISEASES   OF   CHILDREN 

the  regional  lymphatic  nodes,  tuberculous  disease  of  the  bones,  caries  of 
the  petrous  portion  of  the  temporal  bone,  etc.,  can  be  foamd  in  such  cases. 

AFFECTIONS  OF  OTHER  MUCOUS  MEMBRANES 

It  must  be  mentioned  in  relation  to  affections  of  other  mucous 
membranes,  that  some  writers  believe  that  the  chronic  hypertropliic 
inflammations  of  the  nasopharnyx  and  adenoid  vegetations  belong  to 
scrofula.  This  however  cannot  be  admitted.  Chronic  swelling  of  the 
lymphatic  tissues  in  childhood,  wMch  is  especially  prone  to  arise  in 
cases  of  adenoid  proliferations,  is  a  matter  of  such  frequent  occurrence 
in  early  life  in  cases  with  no  suspicion  of  scrofula  about  them,  that  it 
cannot  be  conceived  how  these  two  conditions  can  be  confounded  with. 
one  another.  We  are  here  deahng  with  a  clinically  well  known  aspect 
of  a  disease  which  is  so  successfully  called  by  Heubner  "lymphatism. " 
It  may  briefly  be  mentioned  here  that  the  chronic  sweUing  of  the  lym- 
phatic nodes,  wliich  may  be  placed  in  the  category  with  the  adenoid  vege- 
tations, never  show  tuberculous  characteristics,  while  on  the  other  hand, 
the  tuberculous  nature  of  the  scrofulous  glands  can  hardly  be  doubted. 

THE  SCROFULOUS  GLANDS 

Diseased  cervical  lymphatic  glands  are  very  frequently  observed 
and  most  easily  diagnosticated  (see  Plate  11).  At  first  enlargement  of 
a  single  or  several  glands  takes  place  at  the  angle  of  the  jaw,  before  or 
behind  the  sternocleidomastoid  muscle.  Tliis  swelUng  spreads  more 
and  more  until  an  entire  chain  of  glands  is  involved. 

The  hypertrophy  extends  gradually  and  without  much  pain.  It 
may  become  so  extensive  that  large  tumors  are  formed  on  both  sides  of 
the  neck.  This  disfigurement  along  with  the  chronic  inflammatory 
swelhngs  of  the  face  changes  the  countenance,  giving  it  the  pecuhar 
appearance  of  a  pig  (see  Plate  11).  The  tumors  formed  often  remain 
firm  for  a  long  time,  but  on  the  other  hand  they  frequently  soften  and 
suppurate.  They  may,  through  an  inflammatory  process  become  hard 
and  adhere  to  one  another,  and  to  the  skin.  The  skin  over  the  softened 
glands  becomes  oedematous,  tense,  then  shows  a  bluish  discoloration, 
gets  tliinner  and  at  last  the  gland  discharges. 

Fistulas  may  form  from  which  there  is  a  discharge  of  a  whitish 
flaky  and  purulent  fluid,  mixed  with  curdy  material.  The  fistulas  are 
apt  to  develop  into  ulcers,  which  resemble  tuberculous  ulcers  and  stub- 
bornly resist  treatment.  Similar  phenomena  may  appear  in  all  other 
glandular  regions  although  they  are  less  frequently  found  in  other 
localities. 

The  nature  of  the  glandular  infection  is  still  to  be  considered. 
There  is  hardly  any  doubt  to-day  that  all  scrofulous  glands  are  infected 
with  tubercle  bacilh.     It  is  by  no  means  necessary  that  every  infected 


PLATE  11. 


SCROFULA  235 

gland  should  show  changes  which  are  anatomically  of  a  tuberculous 
nature.  Recent  researches,  particularly  Weichselbauni's,  have  taught 
us  that  virulent  tubercle  bacilH  may  be  present  in  the  medullary  sub- 
stance of  the  swollen  glands.  As  a  matter  of  fact  because  of  the  chronic 
eczema  still  other  bacteria  may  penetrate  to  the  glands  and  cau.se 
abscesses.  However  this  is  only  accidental  and  has  notliing  to  do  with 
the  scrofulous  process  proper.  Of  course  we  see  in  ca.ses  of  chronic 
eczema,  especially  in  eczema  of  the  face  and  scalp  of  nurslings,  consid- 
erable sweUing  of  the  regional  glands ;  but  they  never  manifest  the 
changes  described  above.  They  hypertrophy  and  undergo  a  process  of 
involution,  or  if  they  soften,  they  simply  merge  and  form  a  simple 
glandular  abscess.  Nobody  would  call  such  glands  scrofulous,  for 
they  are  in  every  case  liable  to  infection;  and  tins  is  true  not  only  in 
children,  but  in  adults. 

If  one  would  regard  every  case  of  chronic  eczema  in  young  children 
as  scrofula,  then  he  might  likewise  call  these  glands  scrofulous,  but  the 
whole  conception  of  scrofula  would  be  shifted  thereby. 

DISEASES  OF  THE  BONES  AND  JOINTS 

The  so-called  scrofulous  diseases  of  the  bones  and  joints  most 
assuredly  pertain  to  the  class  of  tuberculosis  and  a  discussion  of  them 
in  these  pages  is  only  a  concession  made  to  the  old  term  "scrofula," 
to  which  those  diseases  are  supposed  to  belong. 

The  Umited  space  wliich  could  be  devoted  in  this  manual  to  the 
subject  of  scrofula  only  permits  a  brief  statement  of  the  general  path- 
ologv  and  symptomatology  of  the  subject.  For  details  we  must  refer 
the  reader  to  the  text  books  on  surgery. 

The  tuberculous  diseases  of  the  bones  nearly  always  appear  as  a 
secondary  disea.se,  originating  through  infection  from  some  existing 
localized  area.  The  structure  of  bone  marrow  is  such  that  tubercle 
bacilli  circulating  in  the  blood  are  easily  arrested,  and  these  subse- 
quently lead  to  the  formation  of  tubercles  in  the  bone  marrow.  The 
tuberculous  granulation-tissue  Hquefies  the  bony  structure,  and  may  lead 
to  necrosis,  or  to  the  formation  of  a  sequestrum.  It  spreads  centrif- 
ugally,  producing  a  state  of  caseation.  This  process  may  extend  periph- 
erally to  the  periosteum  and  cause  a  tuberculous  periostitis.  A  cold 
abscess  may  now  result,  the  process  including  the  overlying  soft  parts. 
Fistulas  may  arise  and  discharge  externally. 

If  the  softening  of  the  bone  is  accompanied  by  enough  resistance 
on  the  part  of  the  periosteum,  then  a  peculiar  spindle-shaped  swelling 
results,  particularly  in  the  phalanges  of  fhe  fingers  which  is  known  by 
the  name  of  spina  ventosa.  Since  the  tuberculous  affections  are  in- 
clined to  remain  in  the  neighborhood  of  the  articulations,  the  extension 
of  the  process  to  the  joints  is  easily  explained.     Primary  involvement 


236  THE   DISEASES   OF   CHILDREN 

of  the  joints  is  rare.  In  the  joint  there  develops  an  exudate  of  a  serous 
or  even  seropurulent  nature.  Gradually  the  synovial  membrane  changes 
to  spongy  granulation  tissue,  which,  penetrating  the  cartilage,  destroj's 
it,  and  detaches  it  from  the  bone.  By  degrees  the  process  spreads  over 
the  soft'parts,  they  swell,  become  oedematous,  the  surface  turning  white 
and  glossy,  and  the  whole  joint  presents  a  spindle-shaped  sweUing 
known  as  tumor  albus  or  white  swelUng. 

Other  diseases  of  the  bones,  spondyUtis  for  example,  are  at  present 
generallj'  separated  from  scrofula  and  for  that  reason  must  be  studied 
elsewhere. 

The  process  in  the  localized  bone  diseases  is  extraordinarily  slow 
and  tedious.  At  first  there  are  no  symptoms,  then  from  time  to  time 
mild  pains,  especially  at  night,  are  felt.  Gradually  these  pains  become 
more  severe,  function  is  disturbed  in  the  beginning  and  finally  is  ren- 
dered impossible.  By  this  time  objective  changes  are  noticeable,  such 
as  have  been  described  above. 

Prognosis. — The  prognosis  of  scrofula  is,  in  general,  favorable. 
The  scrofulous  phenomena  themselves  never  result  in  death.  They 
finally  heal  though  they  may  frequently  last  for  years  without  leaving 
any  marked  functional  disturbances.  Serious  disturbances  may  how- 
ever threaten  the  eyesight,  as  has  been  already  explained. 

But  the  prognosis  assumes  a  different  character  when  we  consider 
the  relation  of  scrofula  to  other  tuberculous  phenomena  and  to  certain 
diseases.  We  must  accustom  ourselves  to  regard  the  scrofulous  indi- 
vidual as  infected  with  tuberculosis,  in  other  words  to  have  an  infec- 
tious disease;  wliich  for  the  time  is  quiescent  and  not  dangerous,  but 
is  apt  at  any  time  to  assume  such  a  role  under  favorable  conditions. 
Such  favorable  conditions  are  unhygenic  surroundings  and  malnutri- 
tion.   A  scrofulous  child  thus  exposed  is  in  great  danger. 

Instead  of  suifering  from  a  comparatively  harmless  form  of  scrofula 
it  may  acquire  a  severe  tuberculous  affection  jeopardizing  permanently 
its  health  and  life.  In  many  cases  re-infection  may  occur  if  the  child  is 
associated  with  a  phthisical  subject. 

There  is  hardly  any  doubt  that  the  form  of  tuberculosis,  whose 
symptoms  are  grouped  under  the  name  of  scrofula,  creates  in  the  organ- 
ism a  certain  sensitiveness  and  susceptibihty  to  re-infection.  Our 
present  biological  knowledge  teaches  us  that  there  is  a  hypersensi- 
tiveness  to  the  virus  in  question,  which  can  be  distinctly  proven  by  the 
reaction  produced  by  the  smallest  doses  of  tuberculin. 

This  inchnation  to  tuberculous  infections  which  positivel}'  exists 
in  scrofulous  children,  teaches  us  of  course,  that  the  prognosis  in  this 
respect  depends  entirely  upon  whether  a  child  is  obliged  to  live  with  a 
person  afflicted  with  phthisis,  or  whether  measures  are  taken  in  time  to 
remove  it  from  its  dangerous  surroundings. 


SCROFULA  237 

The  scrofulous  orgaiiism  is  already  infected  with  the  virus  of  tuber- 
culosis and  if  its  power  of  resistance  is  further  weakened  by  unsanitary 
surroundings  or  by  unhealthy  and  insufficient  food,  the  original  infec- 
tion may  then  assume  more  dangerous  forms. 

The  relation  of  this  statement  to  the  prophylaxis  and  therapeutics 
of  scrofula  will  be  explained  below.  Besides  the  two  etiological  factors 
mentioned,  it  is  stiU  necessary  to  consider  a  few  other  agents  active  in 
the  development  of  severe  cases  of  tuberculosis.  Any  of  the  infectious 
diseases  will  attack  the  scrofulous  organism  more  violently  than  the 
healthy  one;  in  particular  measles  and  whooping-cough  are  to  be  most 
feared.  Both  of  these  infectious  diseases  greatly  favor  the  transfor- 
mation of  a  latent  mild  case  of  tuberculosis  into  a  florid  and  severe  one. 
Consequently  scrofulous  children  who  have  the  misfortune  to  acquire 
either  of  these  two  maladies,  are  to  be  regarded  as  in  great  peril. 

All  the  consequences  resulting  froni  unhygenic  surroundings,  asso- 
ciation with  the  tuberculous,  etc.,  as  mentioned  above,  are  doubly  effec- 
tive under  these  circumstances,  and  in  case  it  is  not  possible  to  remove 
these  pernicious  factors  thoroughly  and  quickly  the  prognosis  is  strongly 
influenced  for  the  worse. 

The  danger  in  the  complication  with  measles  is  present  not  only 
during  its  brief  acute  period,  but  even  after  this  infection  is  past,  so  that 
children  who  have  had  the  measles  under  good  hygenic  conditions,  for 
instance  in  a  hospital,  must  be  still  protected  from  all  injurious  influ- 
ences. Whooping-cough  which  may  last  for  months,  endangers  the 
patient  during  the  entire  period  of  its  course. 

Prophylaxis. — We  can  hardly  discuss  the  prophylaxis  of  scrofula 
because  the  mode  of  its  origin  is  unknown  to  us.  Even  from  the  \iew- 
point  that  every  case  of  scrofula  is  founded  upon  an  infection  with 
tubercle  bacilli,  it  still  remains  unexplained  where  this  infection  has 
taken  place.  If  we  accept  the  theory  of  the  inheritance  of  scrofula — a 
theory  which  according  to  most  recent  researches  cannot  be  rejected — 
then  prophylaxis  is  impossible.  In  the  majority  of  cases  however,  the 
infection  may  possibly  take  place  outside  of  the  uterus,  then  naturally 
precautionary  measures  against  such  infection  may  be  taken. 

The  more  we  are  incUned  to  share  von  Behring's  point  of  view  that 
tuberculosis  acquired  in  the  earhest  period  of  hfe  may  for  a  long  time 
remain  latent,  the  more  we  are  forced  to  believe  that  young  babies 
should  at  once  be  removed  from  any  association  with  the  tuberculous, 
and  that  children  whose  parents  have  active  tuberculosis  should  be 
separated  from  them  as  soon  as  possible.  Among  the  very  poor  the 
removal  of  the  infant  means  at  the  same  time  a  departure  from  unhy- 
gienic surroundings  which  favor  an  infection  ^\ith  tubercle  bacilli  and 
an  outbreak  of  scrofula.  Such  prophylactic  measures  can  very  easily 
be  carried  out  ^\•ith  illegitimate  children.     With  legitimate  cliildren  the 


238  THE   DISEASES   OF   CHILDREN 

problem  is  more  vexatious.  Only  rarely  will  parents  make  the  sacrifice 
of  an  early  separation  from  their  child. 

Attention  must  be  called  to  the  fact  that  sanitary  improvements 
in  the  housing  of  the  poor  are  everywhere  necessary  and  that  scrofula 
and  tuberculosis  could  be  more  successfully  combated  by  such  super- 
vision. In  this  respect  the  prophylaxis  of  scrofula  and  tuberculosis 
so  completely  harmonize  that  a  special  discussion  is  superfluous.  We 
must  refer  the  reader  to  the  article  on  tuberculosis. 

Therapeutics. — Primarily  remove  all  those  pernicious  influences 
wliich  have  been  mentioned  under  prophylaxis.  Narrow,  damp  and 
badly  lighted  dwelhngs  must  positively  be  avoided.  Among  the  poor 
these  precautions  can  but  seldom  be  observed;  and  it  may  in  these 
circumstances  be  advisable  to  place  the  cliild  in  a  well  organized  insti- 
tution. In  addition  to  other  therapeutic  measures,  of  wliich  we  will 
presently  speak,  it  is  evident  that  improved  hygienic  conditions  alone 
will  have  a  far  reaching  influence  upon  the  condition  of  the  suffering  cliild. 

It  is  self-evident  that  only  such  hospitals  for  children  are  consid- 
ered here  as  have  been  built  and  organized  in  accordance  with  the 
principles  of  modern  hygiene.  It  is  a  most  gratifying  fact  that  much 
has  been  done  to  meet  these  demands.  Well  organized  homes  and  hos- 
pitals for  children  have  been  erected,  and  in  the  large  cities  there  are 
ever-increasing  movements  afoot  to  further  these  humane  endeavors. 
Plans  have  been  made  to  send  children  to  the  country  into  the  open 
air:  forest  recreation  retreats,  and  forest  schools  have  been  estabhshed 
for  them. 

Another  requirement  in  the  general  therapeutics  of  scrofula  is 
sufficient  and  suitable  nutrition  for  the  cliildren.  Among  the  laboring 
classes  the  food  is  often  insufficient  and  inadequate,  and  it  is  of  the 
greatest  importance  in  the  treatment  of  scrofulous  children  to  take  good 
care  of  their  nutrition.  It  is  by  no  means  necessary,  nay,  not  even 
desirable,  that  the  diet  of  such  children  should  be  principally  nitrogenous. 
It  is  desirable  that  carbohydrates  predominate:  potatoes,  white  bread, 
green  vegetables,  rice,  etc.,  should  be  given.  Fats  should  be  used  for  the 
scrofulous  as  a  source  of  energy.  I  would  not  advocate  avoiding  or 
Hmiting  it  as  Czerny  teaches.  Fat  is  for  such  children  no  more  injur- 
ious than  for  children  in  general.  The  fat  is  to  be  supplied  in  the  form 
of  butter,  cream,  and  milk.  Undoubtedly  codUver  oil,  that  well-known 
and  popular  remedy,  is  indebted  for  a  great  share  of  its  effectiveness  to 
its  fatty  properties.  Fresh  fruit  should  also  be  included  in  the  cliild's 
dietary.  An  exclusive  milk  diet  is  not  recommended,  in  its  stead  a 
diet  of  good  fresh  vegetables  alternately  with  milk  is  more  suitable  for 
the  scrofulous  patient. 

Further  general  therapeutic  results  are  obtained  by  stimulating 
the  activity  of  the  skin,  the  circulation,  the  respiration  and  the  heart. 


SCROFULA  239 

For  tills  purpose  salt  and  brine  baths  are  very  popular  and  pleasant. 
For  stout,  flabby  children  who  are  frequently  found  among  the  scrofu- 
lous, such  measures  may  prove  of  great  advantage.  By  this  means 
stimulation  or  acceleration  of  metabolism  is  produced,  and  without 
doubt  an  improved  appetite,  an  increased  vivacity  and  a  better  natured 
child  results.  It  is  necessary  however  to  select  carefully  the  children 
suitable  for  this  treatment.  Children  who  have  been  brought  up  in 
wretched  circumstances  and  as  far  as  nutrition  is  concerned  have  known 
nothing  but  want  and  misery,  are  completely  unsuited  for  such  a  plan 
of  treatment.  Such  children  also  require  stimulation  of  their  circula- 
tion, increased  skin  activity  as  well  as  exercise  to  increase  their  powers 
of  resistance,  but  the  measures  used  to  attain  such  results  must  be 
milder  and  comparatively  gentle  in  their  application.  Of  this  we  shall 
speak  hereafter. 

In  general  it  may  be  said  that  brine  and  salt  water  baths  are  used 
too  promiscuously.  The  method  of  gi\'ing  the  bath  is  simple:  from 
two  to  four  pounds  of  salt  are  added  to  the  bath,  which  is  to  have  a 
temperature  from  95°  to  98°  F.  The  child  is  kept  in  the  bath  from  five 
to  ten  minutes,  the  surface  of  its  skin  being  lightlj'  massaged  when  taken 
from  the  bath,  the  patient  must  be  quickly  dried  with  absorbent  towels, 
quite  \igorously  rubbed  and  wrapped  in  warm  blankets.  The  patient 
is  then  kept  in  bed  for  about  an  hour.  If  such  baths  are  not  available, 
similar  treatment  may  successfully  be  resorted  to  by  inunctions  of  soft 
soap.  Every  day  for  five  minute  periods,  the  back  of  the  body  is  rubbed 
with  green  soap  applied  with  soft  cloths,  from  the  neck  to  the  buttocks, 
and  from  the  tliighs  to  the  popHteal  space,  and  then  washed  off  with 
lukewarm  w'ater.  If  the  skin  becomes  irritated,  it  is  well  to  omit  the 
treatment  for  a  few  days.  This  treatment  may  be  continued  from  six 
to  eight  weeks,  and  again  resumed  after  an  intermission  of  from  four  to 
six  weeks.  The  success  attained  by  such  an  inexpensive  and  simple 
method  is  very  often  remarkably  good. 

In  case  we  are  obliged  for  reasons  that  have  been  mentioned  above, 
to  refrain  from  too  active  skin  stimulation,  then  a  gradual  process  of 
hardening  the  body  may  be  employed.  We  begin  with  di-y  frictions  of 
the  whole  body,  once  or  twice  a  day  of  ten  to  twenty  minutes'  duration, 
and  continue  this  process  for  two  or  three  months.  Then  lukewarm 
ablutions  may  be  started,  passing  gradually  to  baths  and  massage  at  a 
cooler  temperature. 

But  we  must  be  extremely  careful  not  to  apph'  any  severe  pro- 
cesses of  hardening  to  feeble  scrofulous  children,  or  to  those  suffering 
from  the  so-called  erethistic  type  of  scrofula.  The  patient's  condition 
often  becomes  much  worse  with  such  inconsiderate  treatment,  and  an 
aggravation  of  the  anaemia  is  the  disagreeable  consequence  of  such  faulty 
management. 


-240  THE   DISEASES   OF   CHILDREN 

To  assist  tliese  therapeutic  measures,  climatic  treatment  can  be 
added  with  great  success.  Sea  baths  are  mostly  in  vogue.  Unfort- 
unately the  right  methods  are  not  always  appUed.  As  regards  the 
feeble,  poorly  nourished  and  anaemic  children,  a  sojourn  at  the  sea- 
shore must  certainly  be  beneficial.  We  must  recollect  that  a  sojourn 
at  the  sea-side  is  unquestionably  of  greater  benefit  than  a  stay  in  an 
inland  region  \\ith  good,  pure  country  air.  For  cliildren  strong  enough 
to  endui'e  the  invigorating  effects  of  sea  climate,  a  residence  on  the 
coast  may  prove  a  powerful  curative  factor.  Of  course  such  a  visit 
must  not  be  too  brief.  Cui'es  lasting  four,  six,  or  eight  weeks,  almost 
always  improve  temporarily  the  general  condition  of  the  patient  but 
rarely  have  a  permanent  effect.  A  sojourn  of  four  to  six  months,  or 
even  a  year  or  more  is  required  to  secure  proper  permanent  results. 
The  winter  seasons  especially  are  too  little  used.  The  fresh  pure  air 
of  the  sea-shore,  a  fairly  even  temperature  \\ith  rarely  an  excessively 
cold  day,  fit  it  particularly  for  a  winter  residence.  In  America,  the 
well-to-do  patient  can  make  a  protracted  stay  at  several  places  on  the 
sea-shore,  during  the  winter  season,  for  there  are  places  which  offer  good 
accommodations  and  even  medical  attendance.  For  the  children  of 
the  poorer  classes,  charitable  organizations  are  beginning  to  make  the 
sea-shore  accessible  and  profitable.  A  rest  at  the  sea-shore  of  four  or 
eight  weeks  at  most  may  result  in  temporary,  but  not  permanent 
benefit.  After  his  speedy  return  to  unhygienic  conditions,  the  advan- 
tages acquired  are  soon  lost  and  the  relatively  expensive  sojourn  has 
done  httle  for  the  child  except  to  indicate  the  road  to  recovery  without 
supplying  him  with  the  means  to  reach  the  goal. 

A  stay  in  the  mountains  is  also  very  beneficial.  For  the  older  and 
stronger  children,  reasonable  winter  sports  may  be  enjoyed  there.  Very 
feeble,  erethistic  children  are  not  well  adapted  for  either  of  these  cli- 
matic changes.  Sparing  the  body,  rather'  than  stimulating  it,  should 
be  the  endeavor  in  these  cases.  Such  children  ought,  if  possible,  to 
spend  the  winter  in  a  mild  climate. 

The  foregoing  remarks  may  suffice  to  throw  some  liglit  upon  the 
subject  of  the  dietetic  and  physical  curative  methods  of  scrofula  in 
general. 

As  to  medicinal  treatment,  there  may  be  given,  codliver  oil,  syrup 
of  iodide  of  iron  and  similar  preparations,  as  guaiacol  carbonate  0.1- 
0.3  Gm.  (l|-5  gr.)  in  powders  several  times  daily,  or  in  codliver  oil 
3  :  200,  a  tablespoonful  twice  daily.  Cresotal  6  to  8  drops  may  be 
given  several  times  daily. 

These  drugs  can  aid  in  securing  the  therapeutic  effect  desired,  but 
can  hardly  accomplish  it  alone. 

Specific  Therapy. — It  has  been  stated  above,  that  nearl}^  all  scrofu- 
lous children  react  to  tuberculin;    and  sometimes  we  see  remarkable 


SCROFULA  241 

improvement,  as  a  result  of  judicious  tuberculin  treatment,  not  only 
in  the  localized  processes,  but  in  the  patient's  general  condition  as 
well.  The  treatment  should  be  used  only  on  patients  that  are  free 
from  fever.  We  begin  with  ^\  to  Jj  mg.  of  old  tuberculin;  or  possibly 
a  little  more,  until  reaction  sets  in;  after  its  absorption,  the  injection 
is  repeated  8  to  14  days  later,  with  doses  as  above  given,  and  continued 
until  reaction  ceases.  Gradually  the  doses  may  be  increased  up  to  1  mg. 
of  the  old  tuberculin.  After  this  a  longer  intermission  may  be  allowed, 
in  order  to  resume  the  treatment  at  the  end  of  a  year  or  later.  In 
Heubner's  clinic  a  few  cases  came  under  my  observation,  showing 
remarkable  improvement,  and  at  a  later  period  complete  cure. 

Eyes. — Calomel  powder,  and  yellow  precipitate  ointment,  once  a 
day.  A  contraindication  is  the  presence  of  new  infiltrates  or  progres- 
sive ulceration.  In  these  cases  apply  atropine  until  the  inflammation 
has  disappeared.  With  larger  ulcers,  and  extensive  corneal  infiltra- 
tions (suppurative  keratitis)  apply  moist  warm  poultices  several  times 
daily  for  one  to  two  hours.  Bandages  are  only  to  be  used  if  the  corneal 
ulcers  are  large,  otherwise  it  is  better  to  do  without  them. 

Eczema  occurring  with  scrofula  is  to  be  treated  in  accordance  \sath 
prevailing  methods.  For  details  on  tliis  subject,  consult  the  chapter  on 
skin  diseases.  Scrofuloderma  is  most  favorably  influenced  by  tuber- 
cuhn  treatment.  Internally,  arsenic  has  been  recommended.  If  ulcer- 
ations are  present,  local  applications  of  balsam  of  Peru,  nitrate  of 
silver,  etc.,  may  be  resorted  to,  e.  g.  in  the  following  form: 

Argenti  nitras 0.3, grs.  v 

Balsam  Peru 3.0, n\,  1. 

Vaseline 30.0, 51. 

Furthermore  scraping  ^vith  a  sharp  spoon,  cauterizing,  or  surgical  treat- 
ment may  become  necessary.  Lichen  scrofulosorum  hardly  needs 
special  treatment.  For  this  disease,  inunctions  with  codhver  oil  are 
indicated. 

Glands  of  the  neck  are  accessible  to  the  knife,  and  it  would  seem 
reasonable  to  remove  localized  tuberculous  tumors.  It  is  true  that 
prominent  surgeons  (Hueter)  have  earnestly  advocated  such  treat- 
ment. However,  the  removal  is  somewhat  dangerous,  for  in  the  first 
place,  the  operation  means  more  or  less  loss  of  blood,  to  wliich  young 
children  in  general  are  very  sensitive  and  particularly  the  scrofulous 
who  are  poorly  nourished  and  antemic.  Secondly,  it  is  extremely  diffi- 
cult to  make  a  complete  removal  and  thereby  avoid  a  recurrence  of 
glandular  hypertrophy  (von  Bergmann).  Thirdly,  experience  teaches 
us,  that  tuberculous  meningitis,  or  general  miliary  tuberculosis  may 
follow  the  extirpation  of  the  glands.  For  this  reason  it  may  be  mo-e 
juchcious  not  to  treat  scrofulous  glands  surgically,  except  those  very 

11—16 


242  THE   DISEASES   OF   CHILDREN 

large  disfiguring  tumors,  which  are  making  firm  pressure  upon  impor- 
tant organs.  Necrosed  glands  and  other  invasions  originating  from 
them,  must,  as  a  matter  of  course,  receive  surgical  treatment. 

Bones  and  Joints. — Extreme  conservatism  is  recommended  here. 
Immobilizing  dressings,  and  Bier's  passive  hyperemia,  must  be  men- 
tioned. For  particulars  on  the  subject  consult  the  text  books  on 
surgery. 


PLATE  12. 


Eruption  of  measles. 


Infectious    Diseases 


MEASLES 

BY 

Dr.  p.  MOSER,  of  Vienna 

TRANSLATED    liY 

Dr.  HAROLD  PARSONS,  M.R.C.P.,  (London),  Toronto,  Canada 


Measles  is  one  of  the  commonest  infectious  diseases  of  childhood. 
Jurgenson  gives  the  eighteenth  century  as  the  date  of  its  definite  recog- 
nition as  an  epidemic  disease.  The  first  important  chnical  and  epi- 
demiological article,  dealing  particularly  with  the  incubation  stage, 
was  by  Panum  in  1846  giving  his  observations  during  an  outbreak  of 
measles  in  the  Faroe  Islands.  Since  that  time  much  has  been  done 
and  pubHshed  from  many  sources  confirming  Pauum's  observations. 
In  the  year  1875  the  interesting  opportunity  again  occurred  to  observe 
the  development  and  spread  of  this  infectious  disease,  in  an  outbreak 
so  severe  that  the  inhabitants  were  cut  ofT  from  communication  with 
the  outside  world. 

Etiology  and  Pathology. — Measles  is  produced  by  an  unknown 
virus  wliich  is  of  a  relatively  evanescent  nature.  It  is  not  possible  to 
carry  the  disease  any  great  distance  by  a  third  person  or  by  means  of 
living  objects.  The  virus  is  short-hved  outside  the  human  body  and 
presumably  can  propagate  only  within  the  human  body.  Whether 
or  not  the  virus  of  measles  can  remain  latent  in  one  who  has  had  the 
disease  is  still  a  question. 

Time  and  Mode  of  Transmission. — The  transmission  of  the  dis- 
ease from  infected  persons  occurs  most  easily  during  the  so-called  initial 
or  prodromal  stage,  and  at  the  time  of  the  rash.  In  the  last  or  stage  of 
convalescence  the  danger  of  transmis.sion  is  not  so  great.  These  two 
first  mentioned  periods  of  measles  are  particularly  well  adapted  to  the 
dissemination  of  the  disease  in  that  during  the  catarrhal  involvement, 
which  predominates  and  in  the  course  of  the  sneezing,  snuffhng,  hawk- 
ing, and  coughing,  the  infecting  organisms  multiply  in  a  most  energetic 
manner,  and  a  still  more  infective  virus  is  produced.  The  greatly 
increased  secretion  assists  in  transmission.  I  recall  a  case,  however, 
admitted  to  the  Hospital  for  a  subsequent  diphtheria,  on  the  fourteenth 
day  after  the  appearance  of  the  rash,  which  infected  children  in  the 
same  ward.     On  the  fourteenth  day  after  the  admission  of  tliis  child  to 

1243 


244  THE   DISEASES   OF   CHILDREN 

the  hospital  the  eruption  of  measles  appeared  simultaneously  in  many 
of  the  patients.  This  case  demonstrates  perhaps,  the  oft-times  strik- 
ing stabiHty  of  the  virus  of  measles  in  persons  recently  infected. 
Measles  very  readily  attacks  those  who  have  not  previously  had  the 
disease.  In  consequence  it  always  occurs  in  great  epidemics  in  thickly 
peopled  areas,  returning  j-ear  after  year,  particularly  in  those  seasons 
in  wliich  catarrhal  conditions  are  most  apt  to  occur.  Conditions  wliich 
bring  together  a  great  number  of  young  persons  are  favorable  to  the 
spread  of  measles,  as  for  example  the  schools,  playgrounds,  children's 
entertainments,  etc.  The  transmission  of  measles  can  result,  (1) 
through  direct  contact  with  an  infected  individual;  (2)  still  much 
more  often  the  conveying  medium  is  air  infected  with  the  poison,  and 
(3)  the  possibility  of  infection  through  the  secretions  of  the  mouth, 
the  nose  and  the  respiratory  tract,  also  the  blood,  lymph,  and  tears, 
conveyed  by  persons,  animals,  or  infected  objects. 

Indirectly  the  desquamation  from  the  skin  may  by  reason  of  its 
infective  nature  contribute  in  the  transmission  of  the  disease. 

The  most  important  carrier  of  infection  in  an  indirect  way  is 
infected  air  which,  vnth  the  help  of  particles  of  dust  or  water  drops, 
serves  as  a  means  for  spreading  the  infection,  although  only  for  a  short 
distance,  as  the  virus  is  short-Uved  in  the  air.  As  a  result  it  happens 
that  epidemics  of  measles  occur  in  the  larger  cities  and  more  thickly 
populated  districts  to  a  greater  extent  than  in  the  more  sparsely  popu- 
lated parts. 

There  are,  it  is  said,  few  persons  who  are  immune  to  measles,  for 
the  predisposition  of  man  to  the  disease  is  particularly  great.  Measles 
is  mostly  acquired  in  cliildhood,  the  period  of  life  which  shows  an 
especially  liigh  grade  of  susceptibiUty.  Adults  experience,  as  in  many 
diseases,  more  discomfort  than  younger  persons;  nevertheless  it  attacks 
them  much  more  lightly.  The  predisposition  to  the  disease  in  later 
life  is  only  apparently  less,  and  I  have  seen  a  woman  sixty-eight  years 
old  with  measles.  The  idea  that  a  lesser  susceptibiUty  to  measles  exists 
in  the  first  six  months  of  life  as  compared  with  the  later  period  of  child- 
hood, is  certainly  not  correct.  Children  under  six  months  of  age  show 
a  diminished  intensity  of  the  symptoms,  sometimes  they  are  only  of  a 
rudimentary  character,  so  that  the  disease  may  be  overlooked,  or  a 
mistake  in  diagnosis  be  made.  They  contract  the  disease  on  exposure 
just  as  readily  as  other  children. 

The  occurrence  of  two  attacks  of  measles  in  the  same  person  is 
rare.  In  most  instances  there  was  a  mistaken  diagnosis,  especially  if 
the  first  infection  should  run  a  milder  course  than  the  second,  but  the 
occurrence  of  a  second  infection  is  not  by  any  means  to  be  denied. 
German  measles,  scarlet  fever,  infective  erythema  and  other  toxic 
erythemata  (those   following   the   use   of   serum   and   such    as   are    of 


MEASLES 


'245 


intestinal  origin)  can  likewise  give  rise  to  error  in  diagnosis.  The 
outbreak  in  an  acute  form,  of  a  fresh  rash  with  associated  catarrhal 
symptoms  occurs  before  the  measles  eruption.  It  occurs  less  frequently 
in  the  above-mentioned  conditions. 

Symptoms. — From  the  day  of  infection  to  the  outbreak  of  the  rash 
is  thirteen  to  fourteen  days.  The  first  signs  of  trouble  are  seen  usually 
on  the  tenth  or  eleventh  day  of  incubation.  I  observed  on  the  sixth  day 
before  the  outbreak  of  the  rash,  in  a  case  of  measles  complicated  wath 
scarlet  fever,  a  slight  rise  of  temperature  and  abundant  Koplik's  spots 
on  the  mucosa  of  the  mouth.  A  long  initial  or  prodromal  period  of 
measles   is  sometimes    found  Fra.  4i. 

in  sick  and  weakly  children. 
This  period,  during  which  the 
disease  reaches  its  full  devel- 
opment, that  is,  from  the  onset 
of  the  symptoms  to  the  out- 
break of  the  rash,  usually  re- 
quires three  or  four  days,  and 
is  marked  by  the  following 
symptoms.  At  first  there  ap- 
pear signs  of  catarrh  of  the 
upper  respiratory  tract  and 
eyes  and  the  child  begins  to 
sneeze.  This  sneezing  may 
soon  pass  off,  but  often  con- 
tinues throughout  the  whole 
initial  period.  Epistaxis  may 
occur  with  the  hyperemia  of 
the  nasal  mucosa,  or  the  ii'ri- 
tation  may  come  on  quietly, 
and  find  expression  in  a  se- 
vere coryza.  The  nasal  secretion  is  at  first  serous  or  mucous,  and  it 
can  also  assume  a  purulent  character.  Severe  catarrhal  changes  in  the 
mucous  membrane  of  the  eyes  are  associated  with  the  coryza  and  are 
shown  by  lachrymation,  photophobia,  and  injection  of  the  conjunctiva; 
the  eyelids  also  show  marked  swelling,  and  adhere  together  in  the 
morning  on  account  of  a  mucopurulent  discharge.  The  separation  of 
the  Hds  is  painful  as  the  dried  discharges  adhere  to  the  edges  of  the 
Uds  and  produce  irritation.  The  signs  disappear  usually  with  those  in 
the  nose.  An  important  part  of  the  catarrhal  symptoms  are  found  in 
the  throat  and  Ijronchi. 

The  first  definite  sign  of  the  approaching  rash  is  a  hypera?mia  of 
the  mucous  membrane  of  the  mouth.  This  is  characterized  by  the 
presence  of  Koplik's  spots.     The  credit  is  due  to  Kophk,  an  American 


Measles  without  conjuactiviti:- 


246 


THE   DISEASES   OF   CHILDREN 


physician,  of  having  drawn  attention  to  this  symptom  which  had  been 
referred  to  in  htcrature,  but  httle  studied  until  now.  Three  or  four 
days,  in  rare  cases  somewhat  longer,  before  the  appearance  of  the  rash 
there  appears  on  the  mucous  membrane  of  the  cheeks  small  bluish 
white,  or  yellowish  white  points,  the  size  of  a  small  pin  head.  They  are 
usually  surrounded  by  a  small  zone  of  reddened  mucosa,  which  has  the 
appearance  of  a  general  reddening  with  the  fine  white  points  upon  it. 
This  hypera^mia  of  the  mucous  membrane  may  be  wanting.  The  wlute 
points  are  mostly  on  the  level  with  the  mucous  membrane,  and  are  less 
noticeable  beside  the  strongly  shining  mucosa.  They  may  be  mistaken 
for  milk  particles  or  fungi.  The  wliite  spots  which  are  composed  of 
Fig.  42.  epitheUum,   detritus  and  bac- 

teria of  the  mouth  adhere 
rather  firmly  to  the  mucosa 
and  on  removal  expose  an 
excoriated,  even  gangrenous 
appearance,  instead  of  a 
smooth  ghstening  mucous 
membrane.  These  are  espe- 
cially numerous  on  the  mu- 
cous membrane  of  the  cheeks 
and  on  the  reflection  on  the 
gums,  and  less  frequently  on 
the  inner  surface  of  the  lips. 
Punctiform  hai-morrhages 
sometimes  occur  as  the  KopUk 
hypersemia  becomes  less,  and 
ulceration  of  the  mucosa  of 
the  cheek  is  found  as  a  result 
of  maceration.  The  Koplik 
efflorescence  usually  begins  to 
fade  when  the  rash  has  reached  its  full  development.  These  form  a 
very  frequent  group  of  signs  associated  with  the  onset  of  measles,  yet 
they  are  often  wanting  in  the  milder  cases,  especially  in  those  occurring 
in  the  first  year  of  Hfe. 

As  a  rule  there  is  a  characteristic  measles  rash  on  the  mucous 
membrane  of  the  mouth.  It  comes  on  suddenly,  lasts  but  a  short  time, 
and  shows  itself  usually  somewhat  later  than  the  Koplik  spots,  situ- 
ated principally  upon  the  soft  and  hard  palate,  with,greater  intensity 
also  on  other  parts  of  the  cavity  of  the  mouth.  It  occurs  in  the  form 
of  pale  or  light  red  irregularly  outhned  streaks  or  spots  between  which 
the  mucous  folUcles  rise.  These  are  swollen  to  the  size  of  a  cherry 
stone,  and  can  be  seen  with  greater  distinctness  on  account  of  the 
pale  color  of  the  mucous  membrane  of  the  palate. 


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Chart  I.    Typical  temperature  curve  in  measles. 


MEASLES 


247 


Concurrently  witli  the  coryza,  irritation  of  the  larynx  and  bronchial 
mucous  membrane  become  evident,  the  early  cough  is  short  and  dry, 
and  the  severe  paroxysms  are  annoying.  With  involvement  of  the  lar- 
ynx the  cough  assumes  a  barking  character,  and  \\ith  still  greater  swell- 
ing of  the  subglottic  laryngeal  mucous  membrane  takes  on  the  character 
of  a  pseudocroup,  wliich  with  the  diagnostic  barking  cough  denotes  a 
greater  or  less  amount  of  laryngeal  stenosis.  This  may  be  sufficiently 
great  to  produce  slight  attacks  of  dyspna-a.  These  laryngeal  changes 
of  the  prodromal  stage  are  however  without  danger  to  life,  in  contrast 
to  those  of  pseudocrou]).  and  croupy  changes  wluch  sometimes  occur  in 
the  period  of  convalescence  and  which  may  prove  a  serious  complica- 
tion. 

Fig.  43. 


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Chart  II.     Long  prodromal  stage. 


Now  and  then  in  small  children,  or  those  weakly  or  tuberculous, 
the  bronchitis  of  the  early  stage  with  its  short  dry  cough  extends  to  the 
smallest  bronclii  and  gives  rise  to  foci  of  bronchopneumonia,  which  in 
its  further  course  is  of  bad  prognosis.  Usually  the  broncliitis  is  charac- 
terized on  auscultation  mostly  by  dry  rales,  and  where  there  is  expec- 
toration it  is  invariably  scanty  and  mucoid.  With  the  outbreak  of  the 
rash  there  is  a  great  increase  of  the  cough,  the  frequency  and  dr3^ness 
of  which  is  distressing  ahke  to  the  patient  and  those  about  liim.  The 
frequency  of  respiration  which  is  the  result  of  lessened  blood  aeration 
and  of  the  high  temperature,  is  increased  to  a  distressing  dyspnoea. 
This  is  made  still  more  harassing  by  the  increased  broncliial  secretion, 
and  numerous  and  various  forms  of  rales.  With  the  fading  of  the  rash 
all  these  respiratory  signs  subside,  either  at  the  same  time  or  shortly 
afterwards. 


248 


THE   DISEASES   OF   CHILDREN 


The  temperature  in  measles  shows  a  fairly  characteristic  curve  as 
the  accompanying  Chart  I,  (Fig.  42),  \n\\  show.  Frequently  in  the  early 
stage  the  elevation  of  the  temperature  may  exceed  39°  C.  (102°  F.).  It 
is  usually  not  of  long  duration  and  gives  way  to  normal  or  subnormal 
temperature  for  one  or  several  days.  With  the  first  appearance  of  the 
rash  the  fever  rises  rapidly  often  to  40°  C.  (104°  F.)  or  over,  and  usually 
assumes  a  continuous  or  remittent  type  until  the  fifth  or  .sixth  day  of  the 
disease  when  it  falls  by  crisis.  It  goes  without  saying  that  this  tempera- 
ture curve  is  subject  to  many  variations  depending  as  it  does  upon  the 
severity  of  the  infection,  the  individual  predisposition  to  temperature 
changes,  and  the  occurrence  of  complications.  It  may  be  therefore, 
that  tliis  two  pinnacle  type  of  curve  in  measles  may,  according  to  the 
height  of  fever  in  one  stage,  take  on  another  form  of  curve;  usually 

however  tliis  particular  type 
will  be  recognizable  in  it  to  a 
greater  or  less  degree.  A 
glance  over  the  accompanying 
temperature  charts  should  make 
the  individual  variations  of  the 
temperature  course  clear. 
Charts  II  and  III  show  the  as- 
sociation of  Koplik  spots  and 
high  temperature.  There  can 
be  a  still  earlier  appearance 
of  the  fever  in  relation  to  the 
Kophk  efflorescence,  so  that  the 
other  prodromal  signs  appear 
first,  and  then  the  meaning  of 
the  rise  of  temperature  is 
difficult  for  the  physician  to  interpret;  in  any  case  a  careful  inspection 
of  the  mouth  should  always  be  made.  With  a  more  protracted  initial 
stage  the  interval  between  the  two  rises  of  temperature  will  naturally  be 
increased,  sometimes  the  rise  of  temperature  occurs  first  with  the  out- 
break of  the  rash.  Elevations  of  temperature  after  the  normal  defer- 
vescence and  after  the  subsidence  of  the  rash  are  mostly  associated 
with  compUcations  (otitis,  stomatitis,  pneumonia,  tuberculosis,  etc.). 
A  late  fever  of  short  duration,  such  as  is  shown  in  Chart  IV  for 
instance,  may  show  no  pathological  reason  for  it.  In  slight  cases,  as 
also  in  nursing  infants,  I  have  often  seen  a  striking  afebrile  course 
in  undoubted  measles. 

When  the  early  stage  has  run  its  course  with  the  symptoms  de- 
scribed, the  eruption  follows  as  the  diagnostic  appearance  of  measles. 
Simultaneously  in  severe  cases  the  catarrhal  manifestations  and  the 
fever  make  their  appearance  in  the  most  intense  form.     The  patient 


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High  temperature  on   one  day  in  a  four 
and  a  half  year  old  boy. 


MEASLES 


249 


Fig.  45. 


shows  great  lassitude,  is  dull  and  delirious,  and  in  small  children  there 
may  be  convulsions.  The  general  condition,  and  the  other  symptoms 
usually  bear  the  closest  relationship  to  the  severity  of  the  rash,  the 
intensity  of  wliich  is  an  index  of  the  severity  of  the  entire  course. 
Very  rarely  there  appears  a  sHght  transient  erythema  on  the  face, 
and  particularly  on  the  neck,  two  or  three  days  before  the  general 
outbreak  of  the  rash,  but  only  three  instances  of  this  rash  have 
come  under  my  observation.  The  rash  spreads  according  to  definite 
rule  over  the  skin,  from  the  tliirteenth  to  the  fourteenth  day  from 
the  beginning  of  the  incubation.  Exceptions  from  the  typical  spread 
or  extension  of  the  rash  are  found  only  in  the  milder  cases. 

The  rash  first  at- 
tacks the  head  and  re- 
gion of  the  face,  where 
the  earhest  appearance 
is  at  the  margin  of  the 
hairy  scalp,  and  the 
region  behind  the  ears, 
and  from  there  it 
spreads  rapidly  over 
the  face  particularly 
the  temples  and  the 
region  of  the  chin.  It 
extends  over  the  neck 
and  downwards  over 
the  upper  arm  and 
trunk,  its  further 
course  is  over  the  fore- 
arms, hands,  the 
thighs,  and  finally  the 
legs  and  feet.  It  fades 
in  the  same  order  as  it  comes.  The  rash  usually  requires  for  its  develop- 
ment and  disappearance  from  three  to  five  days  according  to  its  inten- 
sity, and  leaves  beliind  it  a  pigmentation  of  the  skin  wliich  is  \isible 
for  fourteen  days  or  more.  The  rash  at  its  height  can  cover  the  greater 
part  of  the  skin  surface  at  one  time,  particularly  on  the  second  and 
third  days  of  eruption,  both  the  fading  and  freshly  appearing  rash 
being  from  pale  to  bright  red  in  color,  occasionally  of  a  Uvid  tint.  This 
latter  coloring  occurs  in  the  more  severe  infections,  with  the  onset 
of  pneumonia,  faiUng  heart  with  lack  of  compensation,  and  other 
complications  damaging  to  the  heart  and  lung  functions,  such  as 
myocarditis,  croup,  etc. 

The  rash  often  has  a  pale  appearance  in  nursing  infants,  and  in 
weakly,  debihtated  or  crippled  children.     Usually  the  eruption  varies  in 


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Chart  IV.    Rise  of  temperature  during  convalescence  as  a  result 
of  otitis  media. 


250 


THE   DISEASES   OF   CHILDREN 


size  and  form,  from  the  size  of  a  pin  liead  to  that  of  a  cent,  mostly  irreg- 
ular, and  never  exactly  circular  as  one  often  observes  in  German  measles. 
The  rash  does  not  begin  on  the  surface  of  the  skin,  and  in  its  further 
development  is  usually  of  a  niaculopapular  character,  which  may  easily 
be  felt  by  jjassing  the  finger  over  it.  The  edges  are  not  abrupt  but  fall 
away  gradually.  In  young  children  we  sometimes  find,  as  a  result  of 
greater  infiltration,  that  the  individual  spots  are  raised,  map-like  in 
form  and  with  abrupt  edges  which  can  easily  be  confused  with  other 
forms  of  urticarial  eruption.  The  single  spots  may  run  together  into 
larger  spots  or  patches,  always  leaving  however  greater  or  smaller 
areas  of  healthy  skin  between  them,  so  that  a  mottled,  even  checkered 

Fig.  46. 


Chart  v.     Measles  complicated  with  latent  tuberculosis  of  tlie  broochial  glands.     Boy  four  years  old. 

appearance  of  the  skin  always  occurs,  and  the  designation  in  the  lay 
mind  of  "spots"  or  "spotted  sickness"  is  appropriate.  When  the  finger 
is  pressed  upon  an  old  erythema  the  skin  remains  a  yellow  or  brownish 
color,  especially  in  those  forms  of  measles  in  wliich  the  rash  shows 
blood  infiltration  and  takes  on  a  livid  character.  Effusion  of  blood  into 
the  skin  is  not  uncommon  and  is  absolutely  no  criterion  as  to  the  severity 
of  the  case.  Tliis  can  be  produced  artificially  by  raising  a  fold  of  the  skin 
diagonally  to  its  usual  course  and  pincliing  it.  This  increased  permea- 
bihty  of  the  vessels  of  the  skin  also  exists  in  parts  free  from  the  rash. 
Haemorrhages  occurring  at  the  outset  of  measles  in  sickly,  or  tuberculous 
cliildren,  are  of  bad  omen.  They  occur  in  points  or  patches  and  often 
involve  the  wliole  of  the  abdominal  surface.  Some  instances  are  re- 
corded in  the  literature  of  measles  without  an  ervption  and  the  existence 


PLATE   13. 


a.  Eruption  of  measles  on  leg  and  foot. 

b.  Erythema  infectiopum. 


MEASLES  251 

of  such  cases  cannot  be  doubted,  even  if  we  question  the  diagnosis  and 
the  want  of  knowledge  of  the  observer.  Heubuer  was  able  to  obtain  the 
best  possible  opportunity  to  follow  up  this  matter  in  observations  upon 
brothers  and  sisters.  Undoubtedly  the  best  field  for  clearing  up  such 
caprices  of  the  rash  is  that  of  private  practice. 

In  close  relation  to  the  rash  stands  the  desquamation  of  the  akin, 
which  in  measles  is  an  evanescent  and  sUght  matter  and  often  entirely 
wanting.  Exceptionally,  however,  it  may  appear  in  a  very  marked 
form  and  similar  to  that  of  scarlet  fever.  It  differs  from  this  in  the  fact 
that  the  hands  and  feet  remain  free,  while  on  the  face,  neck,  ti'unk, 
arms,  and  legs  it  is  most  evident.  The  face  is  chiefly  involved  and  shows 
a  marked  peeling.  The  desquamation  is  usually  fine  and  bran-like  in 
character,  but  in  severer  cases  it  may  occur  in  small  flakes. 

As  a  result  of  the  measles  poison,  and  the  skin  changes  induced 
thereby,  there  is  frequently  a  swelling  of  the  lymph-nodes,  chiefly  those  of 
the  cervical  region.  Sometimes  this  swelhng  while  only  sHght,  attacks 
the  whole  lymphatic  apparatus.  The  Uver  and  spleen  are  not  affected 
and  show  no  appreciable  enlargement.  Fairly  regularly  there  is  a  dimin- 
ution of  the  leucocytes,  but  in  the  incubation  stage  a  leucocytosis  is 
observed. 

The  general  condition  produced  by  the  grade  of  infection  and 
combination  of  individual  symptoms  is  dependant  not  only  upon  the 
severity  of  the  illness  but  also  upon  individual  pecuharity.  The  marked 
cerebral  disturbances  (convulsions,  drowsiness,  delirium)  which  appear 
in  many  febrile  diseases  in  infants,  fortunately  are  rarely  seen  in  measles. 
Even  the  initial  stage  shows  certain  disturbances  of  the  general  condi- 
tion, such  as  lassitude,  prostration,  apathy,  headache,  a  sense  of  press- 
ure in  the  eyes,  subjective  sensation  of  light,  irritation  in  the  throat,  a 
sense  of  stoppage  in  the  ears,  symptoms  all  connected  with  the  infec- 
tion and  the  early  catarrhal  condition.  With  the  progress  of  the  disease 
the  drowsiness  is  augmented  and  marked  jactitation  may  appear.  Pains 
in  the  joints,  and  lumbar  pain  is  common  particularly  in  adults.  Loss 
of  appetite,  and  at  the  same  time  rapidly  increasing  tliirst  are  the 
common  accompaniments  of  the  period  of  eruption.  The  general  con- 
dition usually  improves  rapidly  as  the  exanthem  fades,  only  the  lassi- 
tude and  swelUng  of  the  face  are  seen  in  tliis  stage,  just  as  peevishness 
is  the  common  accompaniment  of  the  stage  of  convalescence. 

The  course  of  measles  in  nornud  cases  is  well  defined  and  as  men- 
tioned above  may  be  divided  into  several  stages.  The  whole  period  may 
be  put  down  as  about  three  and  a  half  weeks.  We  differentiate  thus: 
first,  the  period  of  incubation  from  the  beginning  of  infection  lasting 
ten  or  eleven  days,  and  tliis  leads  to,  second,  the  actual  onset  of  the 
disease  as  shown  by  the  outbreak  of  tlie  catarrhal  symptoms.  Tliis  is 
the  initial  or  prodromal  period  and  lasts  two  to  four  days,  so  that  on 


252  •  THE  DISEASES   OF   CHILDREN 

the  thirteenth  or  fourteenth  day  of  infection  we  have  the  period  of 
eruption  characterized  by  the  outbreak  of  the  rash.  The  rash  persists 
three  to  five  days  and  witliin  this  period  it  fades  and  disappears.  This 
period  represents  the  crisis  of  the  disease,  and  the  passing  into  the  stage 
of  convalescence,  which  in  uncomphcated  cases  rapidly  and  immedi- 
ately closes  the  attack.  For  a  week  longer,  on  prophylactic  grounds, 
isolation  precautions  should  be  observed. 

Abnormal  Course,  and  Complications. — These  are  ushered  in  by  a 
fresh  rise  of  temperature  of  a  remittent  or  intermittent  type,  or  no  fall 
may  occur,  a  lower  grade  be  struck,  and  a  continuous  type  of  fever  be 
maintained.  The  most  desperate  form  is  that  described  as  septic  mea- 
sles, wliich  witliin  a  few  days  runs  a  rapid  course  to  a  fatal  issue.  It  is 
probably  the  lessened  resistance  of  the  individual  to  the  virus  of  measles, 
that  accounts  for  the  severe  signs  of  prostration,  the  high  fever  and  the 
acute  course  of  the  disease,  which  toward  the  end  of  its  course  shows  a 
striking  similarity  to  the  toxic  forms  of  scarlet  fever.  It  may  occur  at 
any  time  of  Hfe.  Wliile  the  blood  findings  in  these  fulminating  cases  of 
scarlet  fever  are  always  negative,  in  the  blood  of  septic  measles  on  the 
other  hand  a  double  infection  with  streptococcus  is  found.  The  paren- 
chymatous organs  always  show  marked  degenerative  changes. 

During  measles  and  following  it,  there  are  certain  visceral  comph- 
cations  wliich  must  be  considered.  The  skin  may  first  be  mentioned. 
An  obstinate  eczema  showing  a  variety  of  characters  may  be  associated 
with  measles;  as  for  instance,  fine  nodules  may  develop  and  these  may 
coalesce  and  awake  suspicion  as  to  the  existence  of  a  new  form  of 
measles  rash.  The  rash  is  often  pustular,  pemphigoid,  or  impetiginous 
in  character  where  there  has  been  neglect  in  the  care  and  nursing. 
Ecthyma  with  its  indurated  inflammatory  base  is  also  found  in  such 
neglected  children,  situated  particularly  on  the  buttocks,  and  in  the 
genital  regions.  The  tendency  to  necrosis  of  the  skin  and  mucous  mem- 
branes is  marked  but  fortunately  noma  rarely  develops.  I  once  saw 
in  the  course  of  measles  a  well-marked  dry  gangrene  of  the  prepuce,  yet 
it  was  without  hindrance  to  the  ultimate  recovery  of  the  child. 

A  skin  eruption  only  recently  much  observed  is  nodular  in  charac- 
ter and  tuberculous  in  origin.  The  nodules  are  scattered,  reaching  that 
of  a  lentil  in  size,  brownish  in  color,  sometimes  with  a  blue  discolora- 
tion, often  yellow,  they  are  somewhat  sliiny  in  appearance,  and  the 
infiltration  is  sharply  outUned;  these  are  described  as  tuberculides  (see 
article  by  Leiner  in  Volume  IV.  of  this  work).  They  are  a  definite 
expression  of  tuberculous  infection,  and  are  frequently  seen  in  tubercu- 
lous individuals  in  association  with  measles. 

The  respiratory  tract  is  the  most  frequent  seat  of  cbmpHcations. 
The  measles  virus  alone  or  a  mixed  infection  may  work  serious  damage. 
The  nasal  mucosa  undergoes  inflammatory  changes,  and  the  resulting 


MEASLES  253 

swelling,  particularly  of  the  mucosa,  may  persist  and  interfere  with 
nasal  breathing.  In  cliildren  in  the  first  year  of  life,  as  a  result  of  insuf- 
ficient care,  the  nasal  secretions  excoriate  the  skin  about  the  nostrils, 
and  the  lips,  as  well  as  the  nose  itself,  swell  up  and  become  the  se^t  of 
scrofulous  infiltration.  The  skin  and  mucous  membrane  thus  stretched 
crack,  and  deep  fissures  may  form  which  give  the  patient  great  pain,  and 
in  addition  offer  a  favorable  site  for  the  entrance  of  various  infecting 
organisms.  Commonly  micrococci  are  the  cause  of  these  septic  fissures, 
not  infrequently  it  is  the  bacillus  of  diphtheria.  This  latter  organism 
readily  infects  the  patient  in  the  course  of  measles,  and  it  is  quite 
evident  that  as  a  result  of  measles,  a  distinctly  lessened  resistance  to 
diplitheria  is  shown,  and  the  nose,  throat,  skin,  eyes,  genitals,  but  the 
larynx  in  particular,  are  the  points  of  implantation  of  this  unusually 
rapid  infection.  The  portions  of  the  skin  infected  by  diphtheria  some- 
times show  an  early  and  striking  tendency  to  necrotic  change  which 
may  lead  to  extensive  ulceration. 

Croup  arising  during  measles  is  not  always  necessarily  of  a  diph- 
theritic nature,  yet  this  form  often  occurs.  Sometimes  a  membrane 
forms  in  the  throat,  and  may  extend  to  the  bronchi,  yet  repeated  bac- 
teriological examinations  may  fail  to  demonstrate  the  presence  of  diph- 
theria. This  condition  is  recognized  clinically  by  the  more  yellow  color 
and  loose  adhesion  of  the  membrane  and  shows  micrococci  alone  or  some- 
times influenza  bacilH.  A  pecuharity  that  may  be  mentioned  is  that  in 
spite  of  the  extension  of  the  membrane  into  the  larynx  and  below  it, 
the  throat  may  often  be  free,  or  show  but  little  membrane.  The  signs 
of  croup  can  be  produced  by  swelhng  of  the  mucosa  mthout  the  pres- 
ence of  any  membrane  whatsoever.  Another  cau.se  of  pseudocroup  is 
an  aphthous  inflammation  of  the  mucosa  of  the  mouth  and  larynx, 
moreover  without  the  laryngeal  mucous  membrane  being  affected. 
These  so-called  laryngeal  signs  may  be  produced  by  a  marked  inflamma- 
tion as  a  result  of  an  aphthous  stomatitis  spreading  from  the  throat. 
The  development  of  aphthee  in  measles  and  scarlet  fever  is  especially 
variable  in  character  and  extent.  By  reason  of  the  tendency  to  necrosis 
it  may  produce  extensive  gra5'ish  yellow  discoloration  of  the  mucosa, 
i.e.,  epitheUal  necrosis.  Deeper  losses  of  substance  such  as  are  so 
frequent  in  scarlet  fever,  are  rarely  found  in  measles. 

Apart  from  the  tracheobronchitis  which  commonly  occurs  and 
may  be  of  a  more  or  less  severe  type,  involvement  of  the  lungs  is  the  most 
serious  complication.  Capillary  bronchitis  or  bronchopneumonia  occur 
comparatively  frequently  in  the  first  year  of  hfe.  Objectively  they  are 
evidenced  by  a  sharp  rise  in  temperature  to  40°  C.  (104°  F.)  or  liigher, 
passing  into  a  continuous  form  of  fever,  also  by  rapid  breathing,  dyspnoea 
and  increasing  unrest.  Physical  examination  of  the  chest  confirms  this. 
Frequently  the  disease  is  bilateral,  and  the  area  of  pneumonia  is  diffi- 


254  THE   DISEASES   OF   CHILDREN 

cult  to  localize,  especially  when  it  is  centrally  situated;  small  foci,  espe- 
cially early  in  the  disease,  can  readily  be  overlooked,  particularly  when 
there  exists  at  the  same  time  a  generahzed  broncliitis  of  the  smaller 
tubes,  the  latter  not  uncommonly  causing  atelectasis  in  young  children 
by  reason  of  the  lessened  entrance  of  air  into  the  lungs.  Capillary 
bronchitis  and  a  spreading  croupous  pneumonia  in  the  course  of  measles 
are  most  unfavorable  comphcations. 

In  cases  that  recover,  after  the  disappearance  of  the  fever  and  the 
other  acute  manifestations,  it  generally  requires  several  weeks  before 
the  normal  note  is  found  over  the  situation  of  the  consolidation,  the 
auscultatory  signs  of  consoUdation  disappear  somewhat  earher.  On 
account  of  their  slow  disappearance  Escherich  terms  these  "asthenic 
pneumonia."  They  frequently  raise  a  question  as  to  the  existence  of 
tuberculous  infiltration,  from  which  however  they  are  differentiated 
mainly  by  their  further  course. 

In  persons  with  latent  tuberculosis,  particularly  of  the  broncliial 
lymph-nodes,  a  more  or  less  widespread  tuberculosis  of  the  lungs  may 
develop  with  measles.  This  may  take  the  form  of  a  local  infiltration 
or  a  miliary  tuberculosis  with  a  marked  rise  of  temperature. 

There  is  still  to  be  mentioned  the  acute  necrotic  pneumonia  de- 
scribed by  Heubner.  In  tliis  the  measles  virus  brings  about  an  acute 
necrosis  of  the  lung  tissue  and  in  the  course  of  a  few  weeks  the  produc- 
tion of  extensive  bronchiectases.  The  rash  is  usually  of  a  fleeting  nature, 
fading  rapidly  and  coming  on  long  after  the  prodromal  signs,  and  only 
shortly  before  death. 

The  peculiar  course  of  the  measles  rash  as  well  as  the  acute  pul- 
monary comphcations  may  here  be  described  in  detail. 

Among  the  laity  these  rudimentary  forms  are  spoken  of  as  "  measles 
striking  inward."  After  the  appearance  of  such  a  rash,  lung  comphca- 
tions can  safely  be  surmised.  The  rash  shows  a  pale  or  bluish  discolora- 
tion passing  into  a  deep  cyanotic  appearance  (with  hsemorrhagic  mea- 
sles the  coloration  is  brownish  and  hvid).  The  mucous  membrane  of 
the  hps,  mouth  and  conjunctivie  are  blue.  The  anxious  expression,  the 
movements  of  the  alae  nasi  and  other  signs  of  dyspntjea,  the  great  rest- 
lessness, and  collapse,  complete  the  picture.  It  is  mostly  in  cliildren  in 
the  first  year  of  hfe  that  these  most  severe  and  fatal  forms  are  observed. 

Frequently  the  lung  affection  in  measles  is  brought  about  by  a 
mixed  infection  with  influenza.  In  the  majority  of  systematic  investi- 
gations carried  out  upon  such  forms  of  pneumonia,  the  influenza  bacillus 
was  found  in  the  broncliial  secretions.  Whooping-cough  wliich  readily 
appears  in  association  with  measles,  hkewise  gives  rise  to  acute  and 
chronic  lung  affections,  especially  in  tuberculous  subjects.  They  may 
also  favor  the  outbreak  of  pleurisy,  which  is  mostly  of  the  fibrinous 
variety,  but  may  also  be  serous  or  purulent. 


MEASLES  255 

The  heart  is  seldom  affected  in  measles.  Frequently  during  the 
most  severe  period  of  fever  a  faint  murmur  may  be  heard  for  a  day  or 
two,  without  further  injury  l)eing  discoverable.  The  endocardium, 
myocardium,  and  pericardium  each  may  suffer.  As  a  result  of  measles 
rapid  and  faiUng  heart  action  may  arise,  and  myocardial  changes  are 
the  features  that  remain,  and  by  their  severity  impair  greatly  the 
general  condition. 

A  transient  albuminuria  may  occur  during  the  febrile  period 
without  further  injury  to  the  kidney.  Sometimes  there  is  a  nephritis 
analogous  to  that  seen  in  scarlet  fever.  As  to  causation  these  cases  of 
nephritis  appear  to  be  of  infective  origin,  and  not  infrequently  the 
assertion  has  been  made  that  they  are  produced  by  the  virus  of 
measles,  thus  far  however  they  have  not  been  submitted  to  systematic 
pathological  investigation. 

In  measles  the  frequent  diazo  reaction  in  the  urine  is  an  exndence, 
as  in  typhoid  fever  and  tuberculosis,  of  an  increased  destruction  of  the 
albuminous  bodies,  and  of  a  disturbance  of  tissue  change. 

The  eyes,  which  suffer  an  acute  conjunctivitis  in  the  early  stages 
show  in  the  later  course  of  the  disease  a  tendency  to  chronic  conjuncti- 
vitis and  blepharitis.  This  is  especially  so  in  children  of  a  scrofulous 
tendency  or  as  the  result  of  neglect.  The  conjunctivitis  can  proceed  to 
the  development  of  phlyctenules  and  finally  to  ulceration  with  marked 
photophobia  and  lachrymation  and  as  a  result,  an  extensive  eczema  of 
the  face  may  be  produced.  The  swelhng  of  the  conjunctiva  and  lids 
may  continue  with  intense  purulent  discharge,  in  the  further  course  of 
which  I  have  observed  one  case  of  bilateral  panophthalmitis  which 
apparently  had  its  origin  in  infective  emboUsm,  or  in  infection  from 
without,  the  bacteria  gaining  entrance  through  an  already  poorly 
nourished  cornea. 

The  ears  are  freciuently  the  seat  of  catarrhal  or  purulent  otitis 
media.  This  readily  occurs  in  children  suffering  from  adenoid  vegeta- 
tions, so  soon  as  the  rhinitis  becomes  severe,  and  the  infection  of  the 
nose  and  nasopharynx  extends  into  the  Eustachian  tube.  The  advent 
of  otitis  media  is  announced  by  a  fresh  rise  of  temperature,  often  of  a 
high  grade,  and  usually  of  an  intermittent  type.  The  cliild  becomes 
restless,  complains  of  the  ears  or  of  headache  and  puts  its  hands  to  its 
head.  In  younger  children  opisthotonos  is  frequent  and  mental  dulness 
and  convulsions  commonly  occur.  These  alarming  symptoms  disappear 
with  the  escape  of  the  exudate  through  the  drum-head  into  the  outer 
ear.  With  protracted  retention  of  the  exudate,  or  if  the  suppuration 
becomes  chronic,  carious  changes  can  occur  in  the  bony  structures  of 
the  ear,  in  the  mastoid  antrum  or  of  the  entire  mastoid  process.  The 
objective  signs  of  this  extension  are  redness,  swelhng  and  a^dema  of  the 
skin  over  the  mastoid  process,  pain  on  pressure,  and  protrusion  of  the 


256  THE   DISEASES   OF   CHILDREN 

outer  ear.  If  the  otitis  media  be  one-sided  and  tliere  occur  a  swelling 
of  tlie  lymph-nodes  of  the  same  side  (which  often  occurs  with  otitis) 
then  the  diagnosis  is  clear. 

Moderate  swelling  of  the  lymph-nodes  is  often  present  during  and 
after  mea.sles.  This  sweUing  may  be  general  wliile  the  rash  is  present, 
but  more  frequently  it  is  confined  to  the  cervical  groups.  In  tuberculous 
and  scrofulous  individuals,  particularly  as  a  result  of  eczema,  excoria- 
tions, etc.,  marked  swelhng  of  the  lymph-nodes  may  occur  in  these 
groups,  and  even  proceed  to  suppuration.  The  tendency  to  the  prolif- 
eration of  adenoid  tissue  is  likewise  evident  in  the  region  of  the  pharynx 
and  a  persistent  enlargement  of  the  tonsils  may  be  noted.  More 
frequently  we  find  an  enlargement  of  the  adenoid  tissue  of  the  naso- 
pharynx, which  plays  an  essential  part  in  the  development  of  the' nasal 
and  ear  affections  so  prone  to  arise  after  measles. 

Although  the  lymphatic  apparatus  of  the  intestine,  mainly  the  mes- 
enteric nodes  and  Peyer's  patches  appear  moderately  enlarged,  especially 
during  the  period  of  the  rash,  the  part  played  by  the  intestinal  tract 
is  generally  insignificant.  Nausea,  vomiting,  and  diarrhoea  sometimes 
occur  in  the  initial  and  exanthematous  stages.  The  diarrhoea  may  con- 
tinue until  the  disappearance  of  the  rash  if  care  be  not  taken.  In  young 
cliildrcn  the  condition  is  more  serious  when  the  lower  bowel  is  attacked, 
either  alone,  or  in  association  with  a  former  enteritis,  and  arises  usually 
as  the  rash  is  fading  or  later.  Tliis  lowers  the  resistance  of  the  patient 
and  forms  a  favorable  basis  for  the  development  of  other  infections, 
especially  pneumonia.  The  sharp  outbreak  of  such  an  intestinal  condi- 
tion not  infrequently  leads  to  a  fatal  issue,  by  the  marked  exhaustion, 
intoxication  and  infection.  The  symptoms  are  at  first  those  of  a  moderate 
intestinal  catarrh,  but  soon  the  evacuations  assume  a  mucopurulent 
character,  which  in  turn  give  place  to  movements  of  pure  pus  ■with  an 
admixture  of  blood;  still  later  a  frothy  fermentation  occurs,  the  stools 
have  a  curdled  appearance,  and  a  foul,  sometimes  putrid  odor.  The 
patient  wastes  rapidly,  the  color  of  the  skin  fades  to  a  grayish  tint,  the 
eyes  sink  deep  into  their  sockets,  there  is  marked  prostration,  and  finally 
collapse.  With  tliis  there  is  a  progressively  lower  temperature,  some- 
times the  abdomen  is  much  distended,  very  tender  on  pressure  along  the 
fine  of  the  descending  colon,  and  particularly  so  over  the  sigmoid  flexure. 

The  anatomical  findings  agree  exactly  with  the  chnical  picture  of 
a  severe  dysentery,  in  that  the  large  intestine  shows  deep  gangrenous, 
broken-down  ulcers,  often  of  great  extent.  The  observations  of  Jehle 
as  well  as  the  gradually  increasing  study  of  the.se  intestinal  lesions  point 
to  the  fact  that  we  have  to  do  with  a  secondary  infection  following 
upon  measles,  the  latter  favoring  the  sharp  necrosis  of  the  tissues. 

The  nervous  system  during  the  course  of  measles  shows  no  particu- 
lar  disturbance   apart   from   the   general   condition   already   depicted. 


MEASLES  257 

Exceptionally  there  may  be  mental  dulness  or  convulsions  in  the  initial 
period  or  at  the  time  of  the  rash,  especially  in  children  under  one  year 
of  age.  Severe  inflammatory  changes  though  fortunately  rare  may  even 
occur  in  the  brain  and  its  membranes.  Considering  the  tendency  to 
tuberculous  new  formations  in  association  with  measles,  as  has  already 
been  mentioned,  the  development  of  meningitis  is  to  be  feared.  It  may 
arise  even  after  an  interval  of  one  month,  but  the  other  forms  of  men- 
ingitis, encephahtis,  and  poUomyehtis  are  much  less  frequent. 

The  bones  and  joints  are  but  seldom  involved,  and  here  again  it  is 
chiefly  a  tuberculous  process  that  is  to  be  considered.  Rheumatic 
afTections  which  are  so  frequently  observed  with  scarlet  fever  are  here 
of  rare  nccurreiice. 

Diagnosis. — As  a  rule  the  recognition  of  measles  presents  no  dif- 
ficulty pro\'ided  that  the  disease  follows  the  stereotyped  course,  especially 
in  the  appearance  of  the  rash.  Difficulty  can  arise  in  the  prodromal 
stage  in  the  absence  of  any  trace  of  rash.  The  existence  of  an  epidemic, 
the  points  noted  in  the  history,  and  suspicious  early  symptoms,  such  as 
'attacks  of  sneezing,  snuffling,  coughing,  conjuncti\'itis,  and  shght  rise 
of  temperature  are  presumptive  as  to  the  onset  of  measles.  This  is  made 
a  certaintj'  when  Koplik's  spots  or  red  patches  are  \Tsible  on  the  mu- 
cous membrane  of  the  cheeks  or  gums.  The  search  for  these  must  be 
continued  for  two  or  three  days  on  account  of  their  late  appearance  in 
some  cases.  The  Kophk  spots  are  the  most  important  diagnostic  signs 
in  the  early  stage.  They  are  best  seen  by  diffused  daylight,  less  dis- 
tinctly by  a  glaring  illumination  such  as  direct  sunlight  or  lamphght, 
on  account  of  the  lustre  of  the  mucous  membrane.  Inflammation  of  the 
cheek,  or  particles  of  milk  in  young  cliildren,  can  give  rise  to  error. 
These  latter  can  be  wiped  away,  and  moreover  the  microscopic  exam- 
ination would  show  the  existence  of  oil  globules  or  fungi.  Desquama- 
tion of  the  epithelium  of  the  buccal  muco.sa  and  gums  can  Ukewise  give 
rise  to  mistakes,  but  the  greater  extent  of  these  flakes  and  their  occur- 
rence mainly  on  the  gums,  make  a  differentiation  from  Koplik's  spots 
less  difficult  even  though  they  are  on  the  mucous  membrane  of  the 
cheek,  and  at  the  same  time  not  as  wliite  in  color.  In  German  measles, 
sometimes  punctiform  papules  as  large  as  of  the  head  of  a  pin  are  scat- 
tered on  the  mucosa  of  the  cheek  which  at  first  .sight  resemble  the  Kop- 
lik  spots,  but  they  are  distinguished  from  them  by  their  regular  rounded 
form,  their  sharp  margins,  their  pale  red  color,  and  the  deficiency  in 
the  centre,  distinctly  bluish  white  in  color,  the  result  of  epithelial  necro- 
sis. In  favor  of  mea.sles,  on  the  contrary,  the  KopUk  spots,  when  they 
are  present,  are  an  excellent  differentiating  point,  as  they  occur  in  the 
majority  of  cases  of  measles  and  are  wanting  mostly  in  slight  cases,  and 
then  particularly  in  the  first  year  of  Hfe. 

The  eruption  of  measles  hke  any  other  erythema  causes  great  dif- 

11—17 


258  THE   DISEASES   OF   CHILDREN 

ficulty  in  diagnosis  when  it  is  less  well  defined  and  rudimentary  in  char- 
acter, and  not  accompanied  by  fever.  The  differentiation  from  well- 
marked  German  measles,  more  than  anything  else,  proves  an  obstacle 
to  diagnosis  which  from  a  clinical  standpoint  cannot  be  absolutely 
obviated.  These  can  only  surely  be  distinguished  early  in  the  case 
when  on  the  one  hand  the  Koplik  spots,  and  on  the  other,  the  small 
round  spots  typical  of  the  early  German  measles  can  solve  the  problem 
as  to  diagnosis.  The  more  intense  forms  of  measles  rash  can  lead  to 
confusion  with  other  erythemata.  I  have  met  with  one  such  case  in 
which  there  was  marked  infiltration  of  the  individual  spots,  they  were 
of  a  nodular  form,  hvid  red  in  appearance,  and  particularly  as  they 
stood  in  tliick  groups  together,  several  of  my  colleagues  made  the  diag- 
nosis of  variola.  A  glance  into  the  mouth  suffices  as  a  rule  to  correct 
the  error,  quite  apart  from  the  other  points  of  diagnosis  (in  measles, 
the  preceding  catarrhal  signs,  and  the  intense  redness  of  the  spots, 
in  variola,  a  less  thickly  set  papular  eruption,  oftentimes  leaving  the 
abdomen  free,  and  with  an  early  outbreak  of  pox  upon  the  face,  etc). 

From  scarlet  fever  the  initial  symptoms  of  measles  are  distinguished 
by  the  greater  affection  of  the  alimentary  tract  in  the  former,  the  greater 
angina,  and  the  form  of  the  rash.  The  region  of  the  Hps  and  chin  is 
regularly  free  from  rash  in  scarlet  fever.  An  error  in  regard  to  scarlet 
fever  can  arise  with  the  so-called  confluent  measles,  yet  in  the  general 
grouping  together  of  all  the  symptoms,  and  the  scrutiny  of  all  the  parts 
affected  by  the  rash  one  will  soon  find  some  point  or  another  character- 
istic of  measles.  Measles  and  scarlet  fever  may  however  occur  together, 
and  then  they  form  a  difficult  diagnostic  puzzle. 

Serum  rashes  must  be  mentioned  in  conjunction  with  that  of  measles 
as  they  can  show  a  great  similarity  in  the  skin  and  mucous  membranes. 
The  absence  of  the  Koplik  spots,  the  irregularity  in  the  outbreak  of  the 
rash,  also  the  sequence  in  which  the  several  parts  of  the  skin  are  affected, 
and  above  all  the  fact  of  the  injection  of  the  serum,  will  overcome 
the  difficulty  as  to  diagnosis.  I  have  twice  seen  intense  large  typhoid 
roseola  spots  which  had  a  great  similarity  to  measles. 

Difficulty  may  perhaps  also  arise  with  the  maculopapular  erup- 
tions which  occur  with  the  gastro-intestinal  disturbances  of  nursing 
infants,  especially  when  they  break  out  with  great  severity.  These  are 
intensely  red,  sharply  defined,  and  quite  isolated  spots  about  the  size  of 
a  bean;  they  occur  mostly  on  the  extremities,  and  are,  fike  many  artifi- 
cially produced  erythemata,  characterized  by  the  absence  of  any  change 
whatsoever  in  the  mucous  membranes.  Infectious  erythema  (see  Plate 
13)  as  well  as  erythema  multiforme,  is  characterized  by  its  diverse 
gyrate  outhne,  its  pale  central  portions,  its  locaHzed  occurrence  espe- 
cially upon  the  extensor  surfaces  of  the  extremities.  A  confusion  of 
urticarial  wheals  with  measles,  is  easily  avoided. 


TLATK   U. 


a  3 


MEASLES  259 

Prognosis. — Tliis  is  usually  good  in  strong  healthy  persons  living 
in  good  hygienic  conditions,  even  if  the  attack  be  severe  and  the  gen- 
eral condition  much  affected.  When  the  rash  fades  rapidly  it  is  a  threat- 
ening sign;  this  applies  to  the  adult,  but  still  more  so  to  the  child.  The 
mortality  in  private  practice  is  very  small.  In  Heubner's  polyclinic  in 
Leipzig  it  was  3.1  per  cent.  Jurgenson  in  Tubingen  gives  an  average  of 
6.1  per  cent,  for  20  years.  The  mortality  rates  in  hospitals  alone  are 
not  to  be  compared,  as  here  the  death  rate  is  frightfully  high,  and  in 
many  instances  exceeds  30  per  cent.  This  is  not  to  be  wondered  at 
when  one  considers  that  only  the  poorest  people  send  their  children 
with  measles  to  the  hospital.  These  poorly  nourished,  anaemic  and 
oftentimes  tuberculous  children,  form  with  those  already  in  the  hos- 
pital, and  secondarily  affected  with  measles,  the  sure  prey  of  death. 
That  form  designated  as  "Septic  Measles"  always  leads  to  a  fatal  issue- 
By  reason  of  the  frequency  of  complications  in  the  respiratory  tract, 
children  under  one  year  of  age  furnish  the  greatest  mortahty.  In  one 
epidemic,  Henoch  gives  the  mortality  rate  under  two  years  of  age  as 
55^  per  cent. 

Those  rare  measles  rashes  which  break  out  with  very  high  fever 
and  severe  general  symptoms  in  the  earl}'  stages,  and  which  arc  often 
recognized  only  with  difficulty,  are  unfavorable  from  a  prognostic  stand- 
point. The  U\id  or  brownish  discoloration  of  the  rash  is  to  be  interpreted 
as  pointing  to  the  onset  of  heart  or  lung  comphcations,  and  is  likewise 
unfavorable.  Again,  as  to  prognosis,  as  was  formerly  pointed  out,  the 
temperature  is  worthy  of  note  when  it  does  not  fall  to  normal  as  the 
rash  fades;  tliis  generally  signifies  the  advent  of  comphcations.  Of  all 
the  complications  that  can  occur,  mixed  infection  with  diphtheria  or 
influenza  is  the  most  unfavorable,  as  it  appears  that  those  infected  with 
mea.'iles  show  a  very  much  lowered  resistance  by  reason  of  the  lessened 
production  of  antibodies.  A  most  frequent  and  unfavorable  effect  re- 
sults from  the  advent  of  severe  broncliitis  and  foci  of  pneumonia,  and 
in  consequence  of  existing  or  subsequent  tuberculosis  in  predisposed 
indi^^duals,  likewise  in  rachitic,  aniemic  and  weakly  children,  particular 
caution  is  enjoined  in  predicting  the  further  course  of  the  disease.  The 
tuberculous  lesions  mostly  arise  after  an  interval  of  weeks  or  months  of 
apparent  well-being.  Likewise  one  finds  an  increase  of  the  hemorrhagic 
diathesis  in  those  formerly  predisposed  to  it.  Wliile  purpuric  condi- 
tions following  measles  are  seldom  of  unfavorable  prognosis,  haemo- 
philiacs show  during  measles  grave  progress  in  their  constitutional 
anomaly.  We  may  be  easily  enticed  into  an  unfavorable  judgment  of 
the  course  of  the  disease  by  the  condition  of  the  nervous  system,  as  by 
convulsions,  deUrium  and  stupor.  These  in  all  their  severity,  so  long  as 
they  do  not  last  many  days,  are  of  no  permanent  harm,  as  they  are  of 
an  evanescent  nature,  and  are  not  to  be  interpreted  as  of  bad  prognosis. 


260  THE   DISEASES   OF   CHILDREN 

Of  the  intestinal  disturbances,  only  the  severe  dysenteric  lesions 
are  to  be  feared  as  dangerous  to  life.  The  early  intestinal  disturbances 
are  mostly  slight  and  of  short  duration. 

Prophylaxis. — By  reason  of  the  easy  transmission  of  measles  in 
the  early  stages,  precautionary  measures  to  prevent  the  infection  often 
come  too  late,  and  the  cliildren  who  are  thus  carefully  isolated  from 
the  patient,  share  one  after  another  the  lot  of  their  companions,  unless 
they  possess  a  liigh  grade  of  immunity  against  measles  and  that  is  rare. 
On  this  account  in  many  of  the  villages  of  Southern  Germany  the  cus- 
tom prevails  of  intentionally  putting  the  children  who  have  not  had 
the  disease  into  houses  where  measles  exists,  so  that  by  close  contact 
they  may  contract  it  as  soon  as  possible,  since  it  is  regarded  as  inevita- 
ble and  so  Httle  to  be  feared.  Separation  of  the  members  of  the  family 
from  those  who  have  measles  may  be  regarded  as  useless,  unless  it  is 
done  at  the  very  onset  of  the  initial  stage  (i.e.,  beginning  of  Kophk 
spots)  and  therefore  after  a  very  short  exposure.  On  the  other  hand  it 
is  well  to  take  precautions  against  the  extension  of  the  disease  to  other 
communities,  as  measles  is  transmitted  over  great  distances  with  great 
difficulty,  if  at  all.  School  physicians  together  with  the  teachers,  are 
called  upon,  especially  at  the  time  when  respiratory  catarrh  is  prevalent, 
(considering  the  predisposition  to  measles),  to  take  precautionary 
measures  by  timely  inspection,  in  the  earUest  stage  of  disease,  to  protect 
the  children  who  are  still  unaffected  as  well  as  the  rest  of  the  community. 
This  is  to  be  accomphshed  by  immediate  inspection  from  house  to  house, 
and  by  the  closing  of  the  schools. 

The  cliild  who  has  had  measles  should  remain  away  from  school 
for  at  least  three  weeks  from  the  beginning  of  the  illness.  Tliis  apphes 
also  to  the  children  of  the  family  who  have  been  exposed  but  not  iso- 
lated. If  these  were  immediately  separated  from  the  patient,  and  taken 
to  another  re.sidence,  sixteen  days  quarantine  is  sufficient  before  they 
return  to  school.  Just  as  in  the  case  of  schools,  so  may  other  gatherings 
of  young  persons  during  an  epidemic  serve  as  the  origin  of  infection, 
such  as  at  children's  parties,  play  grounds,  games,  etc.  The  anxiety  as 
to  a  second  attack  is  usually  not  justifiable  when  the  .first  attack  was 
surely  measles.     It  can,  however,  certainly  occur,  but  it  is  very  rare. 

It  is  well  to  sliield  from  measles,  children  under  three  years  of  age, 
those  that  are  weakly,  those  predisposed  to  catarrhal  affections,  those 
whose  brothers  and  sisters  have  died  from  tuberculous  meningitis,  and 
those  predisposed  to  tuberculosis,  or  who  have  already  suffered  from  it, 
or  from  ha?morrhages  or  any  other  malady.  Existing  chicken-pox  and 
whooping-cough  are  said  to  produce  a  heightened  susceptibiUty  to 
measles  though  personally  I  have  not  as  yet  observed  it. 

If  the  disease  is  in  the  incubation  or  prodromal  stage  the  child  is  to 
be  protected  from  taking  cold,  which  vnW  at  any  rate  have  a  therapeutic 


MEASLES  261 

effect.  In  the  stage  of  incubation  the  child  may  be  carefully  taken 
into  the  fresh  air,  but  in  the  prodromal  stage,  the  bed  is  recommended. 
Special  care  for  fear  of  taking  cold  (pseudocroup,  pneumonia),  is  neces- 
sary when  the  prodromal  period  is  protracted. 

Cleanhness  and  other  hygienic  rules  are  the  most  important  pro- 
phylactic measures  during  and  after  the  illness.  The  sick  room  should 
accordingly  have  dry  walls,  and  contain  the  purest  possible  air,  should 
be  large  and  bright,  not  situated  on  the  ground  floor,  and  should  have 
windows  opening  to  the  south  or  west.  The  temperature  should  range 
from  15°-16°  C.  (57°-60°  F.)  the  moisture  of  the  air  must  be  controlled, 
for  we  know  that  with  measles  in  unhygienic  and  badly-ventilated  rooms 
with  deficient  change  of  air,  affections  of  the  respiratory  tract  much 
more  often  develop,  and  run  a  relatively  more  severe  course.  Frequent 
change  of  body  and  bed  hnen,  previously  warmed,  is  advisable,  and  the 
bed  clothes  should  retain  the  heat  well,  but  should  not  be  too  heavy. 
The  daily  batliing  of  the  face  and  hands  with  lukewarm  water  is  regu- 
larly to  be  carried  out.  The  care  of  the  mouth  several  times  a  day  is 
necessary  and  proper,  for  this  in  itself  may  ob\'iate  the  occurrence  of 
the  various  affections  hkely  to  arise  during  the  disease.  I  mention 
these  hygienic  rules,  wliich  speak  for  themselves,  because  it  is  found 
that  even  in  the  better  and  more  intelUgent  classes  of  the  community 
a  real  fear  exists  regarding  the  waslring  of  the  patient  and  the  changing 
of  his  garments. 

In  order  to  guard  against  the  frequent  intestinal  disturbances,  it 
is  well  during  the  disease  to  enforce  a  rigid  diet,  and  strongly  forbid  all 
indigestible  foods,  such  as  breads  made  with  yeast,  raw  fruit,  etc.,  as 
well  as  unnecessary  drinks. 

By  reason  of  the  tendency  to  necrosis  of  the  tissues,  every  form  of 
trauma,  be  it  mechanical  or  thermal,  is  to  be  absolutely  avoideil. 

If  the  period  of  convalescence  has  run  for  eight  days  without  fever 
or  cough,  and  the  patient's  strength  has  sufficiently  recovered  he  may 
be  permitted  to  leave  liis  bed.  Care  must  be  taken  after  measles  on  ac- 
count of  the  lowered  resistance,  especially  of  the  respiratory  tract,  and 
the  patient  should  not  leave  his  room  for  another  eight  days  at  least 
during  the  colder  periods  of  the  year.  The  association  ^^^th  other  cliil- 
dren,  as  before  stated,  may  be  permitted  for  the  first  time  after  the  close 
of  the  period  of  convalescence,  on  the  one  hand,  on  account  of  the  ready 
transmission  of  the  disease  to  them,  and  on  the  other,  because  of  the 
danger  of  the  expo,?ure  of  the  patient  to  some  other  disease.  Particu- 
lar care  should  be  taken  to  avoid  exposure  to  diphtheria  and  whooping- 
cough  to  which  those  convalescing  from  measles  are  known  to  be  very 
susceptible.  It  goes  without  saying  that  one  should  prevent  for  a  long 
time  any  one  affected  with  tuberculosis  from  ha\'ing  any  contact  with 
a  person  that  has  recently  had  measles,  and  on  the  other  hand,  a  measles 


262  THE   DISEASES  OF   CHILDREN 

patient  is  so  disposed  to  tuberculosis  that  he  can  be  said  to  be  safe  from 
danger  of  tuberculous  coniphcations  only  after  months  of  observation. 

The  disinfection  of  the  sick  room  in  uncompHcated  measles  is  an 
unnecessary  procedure,  considering  the  slight  tenacity  of  the  measles 
virus.  Filatow's  suggestion  that  a  two  or  three  days'  airing  of  the  room 
is  preferable  to  troublesome  disinfection  measures,  is  commendable. 

Treatment. — Aside  from  the  prophylactic  measures  which  form 
the  most  important  part  in  ordinary  cases,  and  in  the  absence  of  a  spe- 
cific therapy,  the  treatment  of  measles  is  hmited  to  the  combating  of 
individual  symptoms,  and  the  regulation  of  the  diet. 

In  order  to  lessen  the  intensity  of  the  conjunctivitis  it  is  well  to 
let  the  patient  wear  eye  shades,  or  the  sick  room  may  be  darkened.  I 
have  not  been  able  to  observe  any  more  favoralsle  effect  on  the  course 
of  the  disease  by  the  exclusive  use  of  red  illumination  by  means  of  cur- 
tains or  glass  of  that  color.  For  the  severe  attacks,  one  can  advise 
from  time  to  time  during  the  day,  washing  the  eyes  with  boiled  luke- 
warm water,  or  2  per  cent,  boracic  acid  solution.  The  purulent  crusts 
adhering  to  the  eyeUds  are  best  removed  by  smearing  with  lukewarm 
almond  oil.  Should  phlyctenular  develop  they  are  best  treated  with  1 
per  cent,  yellow  oxide  of  mercury  ointment  or  dusting  with  calomel. 
Applications  of  1-2  per  cent,  solutions  of  blue  stone  produce  a  very 
intense  catarrhal  inflammatory  process.  Ice  poultices  are  not  to  be 
recommended.  The  neighboring  skin  of  the  Uds  may  be  protected  from 
maceration  by  the  tears,  and  resulting  eczema,  by  frequent  smearing 
with  vaseline  or  lanohn.  Diphtheria  of  the  eyes  is  combated  by  serum 
therapy  (3000-6000  units)  and  applications  of  bichloride  of  mercury 
1 : 5000. 

The  coryza,  and  particularly  the  troublesome  sneezing  is  modified, 
and  may  even  be  cured  by  frequent  instillations  of  oil,  or  1-3  per  cent, 
borovasehne  into  the  nose.  For  very  severe  nasal  catarrh,  one  may, 
two  to  three  times  a  day,  introduce  alternately  into  the  nostrils  small 
tampons  of  cotton  soaked  with  1  per  cent,  cocain  solution  and  as  soon 
as  the  passage  is  pervious,  oil  or  vasehne  or  2  per  cent,  yellow  precipi- 
tate ointment  may  be  freely  used.  For  epistaxis,  it  is  sufficient  to  snuff 
up  some  acetic  acid  and  water,  and  if  the  ha?morrhage  be  greater  a  small 
tampon  may  be  introduced  alone,  or,  soaked  in  a  solution  of  adrenahn, 
it  is  sure  to  succeed.  For  severe  nasal  diphtheria,  besides  free  serum 
therapy,  careful  syringing  of  the  nose  with  2  per  cent,  solution  of 
boracic  acid  is  recommended  to  prevent  the  formation  of  membrane. 
The  favorite  procedure  of  blowing  boracic  acid  and  other  powders  into 
the  nose  is  not  to  be  advised,  on  account  of  the  irritation  of  the  mucous 
membrane  which  they  produce,  the  same  apphes  to  the  preparations 
of  menthol.  The  skin  about  the  nostrils  must  be  protected  from  the 
irritating   discharges   by  the  application  of  glycerin,  lanolin,  etc.,  the 


MEASLES  263 

greatest  care  is  necessary  in  the  cleansing  of  the  nose,  and  with  it  a 
frequent  change  of  handkercliiefs. 

For  catarrhal  otitis  media  diaphoresis  is  to  be  produced  by  liot 
drinks  or  sodium  sahcylate,  0.25-2.0  Gni.  (4-30  gr.),  aspirin,  0.15- 
0.5  Gm.  (2-7  gr.)  at  a  dose  are  recommended,  possibly  warm  solu- 
tions of  dilute  acetic  acid  to  the  affected  ear  may  hasten  the  absorption 
of  the  exudate.  In  tliis  as  in  the  purulent  form,  the  severe  pain  will 
be  alleviated  by  the  instillation  of  5  per  cent,  carbol-glycerin.  In  case, 
however,  tliis  does  not  suffice  it  is  necessary  on  account  of  persistent 
liigh  fever  and  the  accumulation  of  pus  to  puncture  the  drum-licad. 
In  very  young  children  (nursing  infants)  tliis  may  be  delayed,  as  the 
pus  readily  escapes  spontaneously,  and  moreover  the  field  of  operation 
is  small  and  vmfavorable.  The  purulent  discharge  from  the  ear  is  best 
combated  by  the  use  of  peroxide  of  hydrogen  and  distilled  water  equal 
parts,  and  if  the  pus  be  very  offensive  and  tliick,  careful  irrigation  with  a 
weak  solution  of  potassium  permanganate,  creoHn,  or  boracic  acid  is 
permissible.  If  the  radical  operation  is  necessary,  let  it  be  done  early, 
as  soon  as  the  purulent  process  extends  to  the  mastoid  antrum.  The 
after-treatment  is  tedious  but  it  gives  excellent  results. 

The  care  of  the  mouth  as  already  mentioned  requires  special  atten- 
tion. The  troublesome  dryness  of  the  mouth  in  young  children  may  be 
overcome  by  frequently  giving  boiled  water,  tea,  etc.,  or  by  carefully 
spraying  the  mouth  with  water.  In  older  children  garghng  with  refresh- 
ing washes  relieves  this  dryness,  or  when  greater  pain  is  present  with 
marshmallow  or  sage  tea.  The  development  of  aphthse  is  treated  by  a 
carefully  arranged  nonirritating  diet,  also  by  frequent  painting  with  a 
solution  of  1-.3  per  cent,  aneson,  or  a  solution  of  copper  sulphate,  and 
eventually  by  touching  the  lesions  with  a  bluestone  pencil.  Internally 
one  may  prescribe  silver  nitrate  (1  to  1000)  a  teaspoonful  at  a  time  in 
severe  and  uncontrollable  cases  (for  instance  in  the  case  of  small  unman- 
ageable children)  (metal  spoons  must  not  be  used).  A  1  per  cent,  solu- 
tion of  potassium  chlorate  may  be  used  with  success  as  a  gargle  in 
aphthous  stomatitis  (this  may  be  used  internally  in  a  solution  of  2  to  5 
grains  to  the  ounce  of  water). 

Noma,  which  is  rare,  should  be  removed  by  the  cautery  or  exci- 
sion. The  frightful  odor  emanating  from  it  can  be  controlled  most 
readily  by  dusting  pure  wood  charcoal  powder  over  the  gangrenous 
parts,  this  may  be  used  alone,  or  combined  with  equal  parts  of  dermatol 
with  the  addition  of  five  or  six  drops  of  oil  of  cade.  An  apphcation  or 
wash  Avith  a  2  per  cent,  solution  of  antinosin  is  also  recommended. 

Should  diphtheritic  deposits  appear  in  the  mouth,  antitoxin  should 
be  administered  as  speedily  as  possilile;  the  same  ajiphes  of  course  in  a 
still  greater  degree  if  the  process  extend  to  tlie  larynx.  As  before  stated, 
one  must  constantly  keep  in  mind  the  fact  of  the  greater  predisposition 


264  THE    DISEASES   OF    CHILDREN 

to  diphtheria  exhibited  by  those  who  have  suffered  from  measles,  the 
particularly  great  loss  of  antibodies  to  diphtheria  demands  a  more 
liberal  administration  of  antitoxin,  5000  to  6000  units  is  to  be  the 
initial  dose  in  undoubted  diphtheritic  croup,  energetic  treatment  it  is 
true,  but  nevertheless  correct.  As  a  further  therapeutic  procedure  we 
think  that  the  immunization  of  all  the  measles  patients  in  the  hospital 
(by  the  injection  of  200  or  300  units  of  antitoxin)  is  to  be  recommended, 
the  danger  of  infection  in  such  patients  lasts  for  several  weeks,  and  it  is 
indeed  a  great  one,  so  that  possibly  the  immunization  may  be  repeated 
at  intervals  of  say  14  days  in  spite  of  the  unpleasant  effects  that  may 
arise  from  such  reinoculation.  The  treatment  of  diphtheria  with  measles 
differs  from  that  generally  followed  in  that  it  must  be  remembered  that 
diphtlieritic  croup  in  the  first  place  gives  rise  more  readily  to  the  de- 
velopment of  foci  of  pneumonia,  and  in  the  second  place  that  it  much 
more  frequently  extends  far  downwards  as  a  descending  croup.  Heart 
tonics,  above  all  infusion  of  digitalis,  0.15-0.5  Gm.  to  70.0  Gm.  (2-7  gr. 
to  2J  oz.),  caffeine  sodium  benzoate,  0.1-0.3  Gm.  (1J-4J  gr.)  given 
daily  internally  or  subcutaneously  as  well  as  the  usual  expectorants 
will  often  overcome  the  first-named  danger.  As  to  the  operative  treat- 
ment of  diphtheria  with  measles,  in  opposition  to  the  usual  course,  I 
would  give  preference  to  primary  tracheotomy,  and  only  in  the  very 
hghtest  cases  of  croup,  would  when  necessary,  suggest  intubation,  the 
frequent  simultaneous  pneumonic  comphcations,  the  tendency  of  the 
croup  to  descend,  and  the  greater  vulnerability  of  the  mucous  mem- 
brane, and,  as  a  result  the  greater  danger  of  ulceration  are  my  main 
reasons  for  tliis. 

Subglottic  laryngitis  or  pseudocroup  in  the  prodromal  stage  of 
measles  presents  no  difficulty  in  the  treatment,  as  it  usually  disappears 
spontaneously  after  the  outbreak  of  the  rash,  moderate  diaphoresis,  fre- 
quent administration  of  warm  drinks  (tea,  lemonade),  inhalations  with 
steam  atomizers,  expectorants,  very  hot  poultices  over  the  throat,  or 
the  inunction  of  mercurial  ointment  suffice.  Counterirritants  such  as 
mustard,  or  one  or  two  leeches  over  the  larynx  may  be  used  in  the  more 
severe  forms,  such  as  may  come  on  in  the  exanthem  or  convalescent 
stages.  In  pseudocroup  also,  in  spite  of  all,  the  question  of  trache- 
otomy or  intubation  must  be  discussed  and  the  decision  as  to  wliich  is 
preferable  has  to  be  made.  Usually  here  intubation  is  to  be  preferred 
particularly  in  \iew  of  the  brevity  of  the  affection. 

The  bronchitis  of  the  early  stages  of  the  illness  is  often  troublesome 
and  is  usually  the  expression  of  the  rash  on  the  broncliial  mucosa,  which 
the  bronchitis  causes  to  disappear.  It  is  always  imperative  to  venti- 
late the  room,  and  that  the  patient  be  not  harmed  by  doing  so  (as  by 
draught).  It  is  further  necessary  to  modify  the  attacks  of  coughing 
with  small  doses  of  codeine.     Expectorants  are  not  called  for  in  the  dry 


MEASLES  265 

form  of  bronchitis,  where  there  is  much  secretion  as  in  capillary  bron- 
cliitis  or  in  the  closing  stage  of  pneumonia.  Ipecac  or  some  other 
expectorant,  will  render  good  service. 

With  pneumonia  or  faihng  heart  it  is  well  to  employ  heart  tonics 
early,  such  as  infusion  of  digitahs,  caffeine,  the  tincture  of  strophanthus, 
or  injections  of  camphorated  oil  may  be  tried.  If  there  be  much  lassi- 
tude and  prostration  alcohol  must  be  used.  Of  course  this  can  be  ad- 
ministered only  in  moderate  quantities,  either  cognac  or  Malaga  wine 
mixed  with  other  fluids  may  be  given  to  nursing  infants  drop  by  drop 
or  to  older  children  by  the  teaspoonful  at  a  time.  It  is  also  well  to 
administer  a  Hght  white  wine  in  the  form  of  a  wine  soup.  It  is  quite 
inexcusable  on  the  grounds  of  temperance  to  exclude  alcohol,  that  great 
saver  of  tissue  waste,  from  the  physician's  armamentarium,  even  if  its 
efhcacy  is  accompUshed  only  at  the  cost  of  inliibiting  the  action  of  the 
vagus  nerve.  The  harmful  effects  of  alcohol,  as  with  any  other  medicines, 
arise  only  from  the  long  continued  consumption  of  large  quantities. 

The  nausea  produced  by  medication,  as  often  formerly  occurred  for 
instance  in  capillary  broncliitis  or  in  the  closing  stage  of  pneumonia, 
should  on  account  of  the  heart  always  be  avoided  with  the  utmost 
caution. 

An  important  part  of  the  treatment  in  the  bronchitis  and  pneu- 
monia of  measles  is  hydrotherapy.  As  to  whether  tliis  form  of  treat- 
ment can  cut  short,  or  form  a  barrier  to  the  disease  is  very  doubtful. 

The  changes  in  the  rash  (Hvid  discoloration  and  washed  out  ap- 
pearance) already  spoken  of,  such  as  often  appear  in  the  course  of  severe 
heart  and  lung  complications,  and  called  by  the  laity  "relapsing  measles," 
the  popular  mind  readily  ascribes  to  the  hydrotherapy.  Unfortunately 
at  times  the  lung  conditions  increase,  in  spite  of  scientific  treatment 
mostly  however  it  is  where  the  activity  of  the  heart  and  the  general 
condition  have  been  overlooked,  and  even  the  most  serious  symptoms 
(as  for  instances  cyanosis  of  the  mucous  membranes  and  the  peripheral 
parts  of  the  body  as  well  as  coldness)  remain  unnoticed. 

A  cool  pack  to  the  nape  of  the  neck  (a  towel  wrung  out  of  water 
at  25°  to  28°  C.  (77°  to  82°  F.)  and  covered  with  a  larger  bath  towel), 
may  in  many  cases  not  only  reduce  the  temperature,  but  by  it  the 
general  condition  may  be  improved,  and  pain  and  difficulty  in  breath- 
ing alleviated.  By  three  appUcations  at  intervals  of  twenty  minutes  a 
favorable  lowering  of  temperature  will  readily  be  obtained,  whilst  in 
other  cases,  where  the  fever  is  not  so  liigh,  but  the  other  symptoms  are 
troublesome,  a  longer  continuance  of  the  ajipHcations  (two  to  four 
hours)  is  desirable.  When  these  are  to  be  frequently  repeated  a  pre- 
vious anointing  the  skin  of  the  part  is  well  as  a  preventative  against 
eczema.  If  dyspncea  and  prostration  increase  and  there  be  deficient 
expectoration,  warm  baths  (35°  C;  95°  F.)  with,  a  cooler  douche,  carefully 


266  THE   DISEASES   OF   CHILDREN 

used,  are  often  beneficial.  Hyperaemia  and  diaphoresis,  and  thereby  a 
relatively  greater  radiation  of  heat  from  the  skin  can  be  increased  by 
mustard  baths  (50-100  Gm.  per  bath)  or  as  Heubner  suggests,  by 
wrapping  in  mustard  water  (^  kilogram  to  IJ  litres  of  warm  water).  While 
these  means  are  employed  in  weakly  and  reduced  cliildren,  I  should 
advocate  blood  letting  in  the  form  of  leeches  or  venesection  where 
one  has  to  deal  with  strong  well  nourished  children,  in  preference  to  all 
other  methods. 

The  inhalation  of  oxygen,  in  many  cases,  especially  in  severe 
broncliitis,  brings  about  an  improvement  of  the  subjective  symptoms 
and  a  lessening  of  the  respiratory  frequency. 

The  tuberculous  affections  of  the  respiratory  tract,  glands,  brain 
and  skin,  etc.,  must  be  combated  by  sufficient  nourishment  under 
favorable  chmatic  and  hygienic  conditions,  with  mental  and  physical 
rest.  Creosote  and  its  derivatives  may  be  administered  in  moderate 
quantities.  With  local  tuberculous  processes  iodine  and  the  inunction 
treatment  are  to  be  employed  before  the  time  for  surgical  interference. 
For  the  simple  inflammatory  adenitis  the  appHcation  of  moist  warm 
poultices  of  five  to  ten  per  cent,  of  ichthyol  ointment  are  successful. 

For  the  intestinal  catarrh,  a  restricted  diet  is  sufficient  for  a  cure 
at  the  beginning  of  the  disease  and  yet  will  sustain  the  patient  during 
the  febrile  period  with  his  loss  of  appetite.  Apart  from  dietetic  measures 
the  later  severe  coHtis  is  combated  by  frequent  irrigations  with  warm 
boiled  water  at  40°  C.  (104°  F.)  either  alone,  or  with  the  addition  of 
tannin  1  per  cent,  or  acetic  alum  1  to  2  per  cent,  or  with  50-100  c.c. 
of  1  per  cent,  silver  nitrate  solution,  Ukewise  by  giving  the  bismuth 
preparations  by  mouth.  If  bacteriologically  Kruse  dysentery  be  diag- 
nosed one  must  not  hesitate  in  the  administration  of  a  corresponding 
serum.  Much  may  be  done  in  a  prophylactic  way  to  prevent  these 
intestinal  troubles,  if  from  the  onset  of  the  disease  undue  irritation  of 
the  intestinal  mucosa  is  avoided  by  a  sensible  and  not  an  immoderate 
administration  of  medicines  (digitaUs  and  alcohol,  etc.),  and  a  light 
diet.  The  diet  should  be  mainly  liquid  (tea,  soup,  milk,  cocoa),  wluch 
may  with  improving  appetite  be  changed  to  soft  easily  digested  foods, 
(sago,  tapioca,  and  eventually  minced  meat). 

The  nervous  symptoms,  as  dulness,  convulsions,  dehrium,  headache 
and  jactitations  are  to  be  treated  by  cold  appUcations  to  the  head  or  gen- 
eral wet  packs  at  a  temperature  of  27°  C.  (80°  F.)  mustard  packs  or 
mustard  baths,  likewise  the  administration  of  sodium  bromide,  0.15  to 
1.0  Gm.  (2  to  15  gr.)  or  pyramidon,  0.1  Gm.  (li  gr.)  may  be  tried. 
In  emergencies,  when  the  cerebral  signs  do  not  abate,  spinal  puncture 
is  highly  recommended  as  a  means  of  reheving  the  brain  of  the  over 
accumulation  of  cerebrospinal  fluid.  Prostration  and  the  pains  in  the 
Umbs  can  be  reheved  by  the  hmited  administration  of  alcohol  (Malaga 


MEASLES  267 

wine,  cognac),  also  by  rubbing  with  dilute  acetic  acid  or  some  alcoholic 
solution,  and  internally  some  sodium  salicylate  or  aspirin. 

Sharp  rise  of  temperature  the  result  of  measles  and  its  compUca- 
tions  is  best  influenced  as  already  stated  bj^  hydrotherapeutic  measures. 
Where  these  are  unsuccessful  small  doses  of  aspirin,  quinine  or  aristochin  . 
may  be  given. 

Sometimes  there  is  irritation  of  the  skin,  which  is  best  relieved  by 
sponging  the  parts  with  diluted  alcohol  or  by  the  use  of  saUcylic  acid 
or  menthol,  also  by  some  protective  covering  such  as  oil  or  a  dusting 
powder.  Sponging  is  preferred  particularly  if  there  is  desquamation 
of  the  skin  at  the  time. 

The  eczema  and  other  skin  changes  follo\\ing  measles  require  effi- 
cient treatment  which  need  not  be  discussed  here. 

At  the  end  of  the  attack  of  measles  and  its  associated  troubles  the 
patient  should  take  particular  care  of  the  skin  by  taking  one  or  two 
full  warm  baths  before  lea^^ng  bed.  The  patient  may  leave  his  bed 
eight  days  after  the  subsidence  of  the  fever,  generally  after  another 
eight  days  he  may  be  allowed  to  go  out  of  doors,  but  the  time  of  year 
and  the  state  of  the  weather  will  decide  this. 


SCARLET  FEVER 

Dr.  C.  v.  PIRQUET,  of  Vienna,  and  Dr.  B.  SCHICK,  of  Vienna 


PRIMARY  SYMPTOMS  OF  SCARLET  FEVER,  by  Dr.  C.  v.  PIRQUET 

TRANSLATED    BY 

Dr.  ISAAC  A.  ABT,  Chicago,  III. 


INTRODUCTION 

Historical. — Scarlet  fever  is  a  disease  which  has  been  endemic  in 
Europe  for  centuries.  The  separation  of  the  disease  from  other  infec- 
tious exanthemata,  especially  from  measles,  occurred  in  the  17th  cen- 
tury (Sydenham).  It  remained  for  the  19th  century  to  differentiate 
diphtheria,  first  clinically,  later  bacteriologically  from  the  pharyngeal 
effects  of  scarlet  fever. 

The  etiology  of  scarlet  fever,  even  to-day,  is  not  clear.  It  is  true 
that  streptococci  are  almost  constantly  found  in  the  organs  of  patients, 
but  the  question  whether  they  should  be  regarded  as  etiological  agents 
or  simply  as  a  mixed  infection  has  engaged  numerous  workers  since 
1885.  Upon  the  significance  of  streptococci  depend  the  efforts  of  the 
last  years  to  influence  scarlet  fever  therapeutically  by  a  streptococcus 
serum. 

GENERAL  CLINICAL  PICTURE 

Typical  Case. — Franz  H.  (Fig.  47),  eleven  years  old,  at  noon  pre- 
sents sore  throat  and  vomiting;  a  few  hours  later  a  rash  appears  on  the 
body,  which  rapidly  extends  to  the  extreinities.  Temperature  at  7 
p.  M.,  38.7°  C.  (101.6°  F.);  later  in  the  evening,  39.5°  C.  Next  morning, 
38.4°  C.  (101.1°  F.).  On  admission  there  is  a  rash  over  the  entire  body, 
which  from  a  distance  seems  to  be  a  faint  reddish  confluent  erythema; 
on  clo.ser  examination,  however,  it  is  found  to  be  made  up  of  small, 
closely  placed  spots.  Each  .spot  is  2  to  3  mm.  in  diameter,  poorly  de- 
fined, and  not  raised  above  the  surface  of  the  skin.  The  spots  disap- 
pear completely  on  pressure.  The  rash  is  better  marked  on  the  inner 
aspect  of  the  extremities  than  on  the  outer.  The  region  of  the  mouth 
is  pale,  and  is  sharply  separated  from  the  red,  swollen  cheeks  as  a  clear 
triangle  of  yellowish  hue.  The  Ups  are  cherry  red;  inspection  of  the 
oral  cavity  shows  slight  uniform  reddening  of  the  hard  palate  and  the 
buccal  mucous  membrane.  The  soft  palate  and  the  uvula  are  of  a 
deeper  red;  the  tonsils  are  prominent  and  covered  by  a  yellow  follicular 
exudate.  The  dorsum  of  the  tongue  has  a  white  fur.  At  the  angles  of 
the  jaws  there  is  on  each  side  a  somewhat  tender  gland  as  large  as  a 

268 


SCARLET  FEVER 


269 


bean.  The  child  is  cheerful  and  complains  only  of  pain  in  swallowing. 
The  nose  is  dry  and  admits  air  freely.  There  is  notliing  abnormal  about 
the  eyes,  ears,  lungs  or  heart;  only  the  heart  action  is  rapid  (168). 
Evacuation  of  the  bowels  occurs  after  the  administration  of  castor  oil; 
the  stools  are  normal.  Urine  contains  no  albumin.  The  spleen  is  not 
palpable;  the  liver  extends  1  cm.  below  the  costal  arch. 

Diagnosis. — This  is  a  typical  case  of  scarlet  fever.    The  acute  on.set 


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Typical  temperature  curve  in  scarlet  fever. 

of  the  disease  with  vomiting  and  sore  throat  in  the  midst  of  perfect 
health,  the  rapid  development  of  the  rash  and  its  typical  spread  and 
character,  permit  a  positive  diagnosis.  Measles  may  be  excluded,  be- 
cause the  conjunctivae  are  pale,  the  child  does  not  sneeze  or  cough, 
and  the  rash  does  not  involve  the  region  of  the  mouth.  Opposed  to  the 
diagnosis  of  a  simple  follicular  tonsillitis  are  the  dark  red  discoloration 
of  the  pharynx  and  especially  the  simultaneous  appearance  of  a  rash. 
The  rash  is  too  dense  and  too  bright  red  for  rubella  and  the  fever  is 
too  high. 


270  THE   DISEASES   OF   CHILDREN 

Pathogenetic  Connection  of  the  Symptoms. — From  the  fact  that 
sore  throat  was  the  earhest  symptom  and  that  tliis  was  followed  by 
signs  of  a  general  infection,  we  should  say  that  the  tonsils  were  the 
primary  localization.  This  will  become  more  apparent  later,  when  we 
compare  it  with  cases  in  wliich  the  primary  infection  occurred  else- 
where (extrabuccal  infections)  (p.  277).  As  in  septic  and  puerperal 
processes,  in  diphtheria,  tetanus  and  syphilis,  we  must  distinguish  the 
primarj^  focus,  with  its  regional  manifestations,  from  the  results  of  the 
general  infection. 

Throat. — The  tonsil  is  a  site  of  predilection  for  the  primary  infec- 
tion of  scarlet  fever.  In  mild  cases  the  disease  appears  as  redness  and 
swelling  of  the  tonsils' alone;  in  moderate  cases  as  a  follicular  tonsiUitis; 
in  very  severe  forms,  the  tonsil  and  surrounding  tissue  may  undergo  a 
superficial  or  deep  necrosis  (gangrenous  angina).  Streptococci  are  always 
found  on  or  in  the  tonsils. 

From  the  site  of  earliest  involvement,  the  infective  agents  spread 
through  the  lymphatics  to  the  regional  lymph-nodes;  also,  by  direct 
extension,  to  the  mucous  membrane  of  the  nose  and  nasopharynx, 
from  which  they  maj^  pass  through  the  Eustachian  tube  into  the  middle 
ear.    The  primary  infectious  complications  result  in  this  way. 

Nose. — In  the  case  presented  we  note  that  nasal  respiration  occurs, 
and  that  the  nose  is  dry.  An  infection  would  have  shown  itself  by  a 
mucopurulent  discharge,  or  by  obstruction  of  the  nose. 

Ear. — The  ear  drums  are  pale.  If  the  nose  is  not  involved,  it  is 
probable  that  the  middle  ear  is  also  uninvolved.  Still  it  is  advisable  to 
examine  each  case  with  the  ear  speculum. 

Lymph-nodes. — We  see  by  the  slight  enlargement  of  the  lymph- 
nodes  at  the  angle  of  the  jaw  that  the  infection  has  passed  along  the 
lymphatics. 

We  group  all  of  the  phenomena  which  depend  upon  the  passage 
of  the  disease-producing  factors  to  the  neighborhood  of  the  primary 
site,  as  infectious;  the  other  constitutional  symptoms  (initial  vomiting, 
fever,  tachycardia  and  rash)  are  classed  as  toxic.  We  believe  that  the 
latter  depend  upon  the  reaction  of  the  organism  to  the  bacterial  toxins, 
produced  by  the  germs  at  the  site  of  the  primary  infection  and  its  sur- 
roundings. It  will  not  be  pos.sible  to  decide  whether  tliis  separation  of 
symptoms  is  justifiable,  until  the  etiological  agent  of  scarlet  fever  is 
definitely  determined.  At  any  rate,  it  indicates  our  present  knowledge 
and  serves  as  a  good  foundation  for  our  cHnical  conception  of  the  disease. 

Incubation  Period. — The  time  between  the  introduction  of  the 
infection  and  the  first  symptoms,  the  period  of  incubation,  appears  to 
vary  a  great  deal,  and  sometimes  to  be  less  than  twenty-four  hours. 
(see  p.  273).  In  this  particular,  scarlet  fever  differs  decidedly  from 
the  other  acute  exanthemata.     In  measles,  chicken-pox,  smallpox  and 


SCARLET  FEVER  271 

rubella,  the  time  of  incubation  is  much  longer  and  usually  well-defined. 

No  Pronounced  Prodromata. — The  period  of  prodromal  symptoms 
in  scarlet  fever  is  even  less  definite  and  shorter.  Between  the  initial 
vomiting  and  the  appearance  of  the  rash  often  only  a  few  hours,  rarely 
more  than  a  day,  elapse.  Hence,  we  consider  the  description  of  a  pro- 
dromal stage  as  unnecessary  and  speak  only  of  initial  symptoms.  A 
history  of  illness,  dating  back  several  days,  has  no  connection  with 
scarlet  fever.  It  often  occurs  that  children  have  a  tonsillitis  and  general 
malaise  for  several  days  preceding  the  appearance  of  the  rash.  We 
beUeve  that  in  these  cases  the  preceding  sore  throat  was  not  specific 
and  sim])ly  prepared  the  ground  for  the  scarlatinal  infection. 

Initial  Symptoms. — The  typical  initial  symptoms  are  sore  throat, 
vomiting,  lassitude,  and  fever. 

Rash. — In  our  case  vonaiting  began  at  noon;  at  7  p.  m.  the  exan- 
them  is  alread)'  distinctly  visible.  The  same  evening  the  temperature 
rises  to  39.-5°  C.  (103°  F.).  The  rash  usually  appears  first  on  the  chest 
(see  Plate  15),  at  the  same  time  the  face  is  swollen,  and  the  cheeks  are 
flushed.  This  swelling  and  flushing  are  sometimes  so  characteristic 
that  from  them  alone  one  may  make  a  tentative  diagnosis.  Later, 
the  rash  spreads  rapidly  to  the  shoulders  and  passes  along  the  inner 
aspect  of  the  arm  to  the  hands.  It  does  not  involve  the  outer  aspect 
until  later,  and  then  less  intensely.  On  the  lower  extremities  the  inner 
surface  of  the  tliighs  first  becomes  injected,  later  the  other  parts  of  the 
skin.  The  rash  is  often  feebly  developed;  if  so,  the  spots  appear  only 
along  the  inner  surface  of  the  arms  and  tliighs  and  in  the  genital  tri- 
angle. It  also  occurs  that,  even  when  the  rash  is  intense  on  the  body, 
the  face  appears  pale,  even  anemic.  Therefore,  it  must  be  made  a  rule 
to  examine  the  whole  body  of  ever}-  cMld  with  a  follicular  tonsilhtis 
or  a  marked  reddening  of  the  soft  palate.  When  the  rash  has  spread  to 
the  hands  and  feet — usually  witliin  twenty-four  hours — it  increases  for 
a  time  in  intensitj',  and  in  many  places  a  uniform  erythema  may  develop 
by  confluence  of  the  small  numerous  bright  red  spots.  This  shows  the 
typical  scarlet  color,  to  wliich  the  disease  owes  its  name. 

Icteric  Coloring  and  Pigmentation. — In  typical  cases  the  rash  re- 
mains at  its  maximum  till  the  third  day  of  the  disease.  At  first  it  dis- 
appears completely  on  pressure;  only  over  the  abdomen  a  pale  yellow 
tint  persists.  The  cause  of  this  color,  wliich  may  be  very  distinct  in 
severe  cases,  is  not  known.  During  the  later  days  of  the  disease,  the 
skin,  on  pressure,  shows  another  pigment,  which  is  proportionate  to 
the  intensity  and  duration  of  the  rash.  It  is  pale  brown  and  is  to  be 
regarded  as  blood-pigment. 

Disappearance  of  the  Rash. — The  rash  disappears  in  the  same  order 
as  it  appeared.  The  color  of  the  trunk  fades  earliest:  later  the  extrem- 
ities.    The  exanthem  cannot   be  interpreted  as  simply  a  hyperaemia, 


272  THE   DISEASES   OF   CHILDREN 

because  of  the  slight  diffuse  swelUng  of  the  skin  at  the  height  of  the 
rash,  the  deposit  of  pigment,  and  finally  because  it  results  later  in  an 
exfoliation  of  the  entire  outer  layers  of  the  epidermis. 

Desquamation. — Desquamation  varies  considerably  in  the  time  of 
its  beginning;  it  does  not  occur  in  every  case.  Its  extent  is  usually 
proportionate  to  the  intensity  of  the  preceding  rash.  In  the  second 
week,  on  the  average,  the  first  signs  of  peehng  begin  on  the  neck  and 
chest,  on  the  inner  surface  of  the  thighs,  on  the  buttocks  and  back; 
finally — often  only  in  the  fourth  to  sixth  weeks — new  skin  forms  on  the 
palms  of  the  hands  and  soles  of  the  feet.  Here  the  tliick  skin,  unlike 
that  of  other  parts,  is  loosened  in  large  lamella^.  Glove-Uke  casts  (Plate 
IS)  are  easily  recognized,  but  occur  rarely.  It  is  more  difficult  to  recog- 
nize the  milder  but  just  as  characteristic  forms  of  desquamation. 

Enanthem. — ^A  change  in  the  mucous  membrane  of  the  mouth,  the 
enanthcm,  corresponds  to  the  exanthem.  This  occurs  even  in  cases  in 
which  the  tonsils  are  not  primarily  involved.  The  mucous  membrane 
becomes  diffusely  reddened.  Koplik  spots  never  occur,  as  in  measles. 
Desquamation  occurs  in  the  mouth  as  well  as  on  the  skin.  But  the 
process  is  much  more  rapid,  probably  on  account  of  maceration  of  the 
moist  surface.  The  gums  earl}'  have  a  milky  appearance;  desquamation 
of  the  tongue  goes  on  more  distinctly  and  more  rapidly.  In  quite  early 
cases  the  tongue  is  red  and  slightly  coated.  The  coating  becomes  thicker 
on  the  first  days;  later  it  is  cast  off,  first  at  the  tip,  then  at  the  root. 

Strawberry  Tongue  (see  Plate  14). — We  designate  as  "strawberry 
tongue"  one  which  has  become  clean,  is  still  bright  red  and  swollen, 
and  whose  papilke  are  infiltrated  and  very  prominent.  The  straw- 
berry appearance  is  not  an  initial  symptom,  but  appears  on  the  third 
to  sixth  day,  and  disappears  gradually  in  the  course  of  the  next  two 
weeks.  First,  the  color  fades,  and  then  the  papillae  diminish  in  size. 
This  symptom,  together  with  the  existence  of  a  rash,  is  pathognomonic 
of  scarlet  fever:   alone,  it  is  insufficient  (p.  283,  Marie  E.). 

The  character  of  the  temperature  and  pulse  should  be  considered 
among  the  constitutional  symptoms. 

Fever. — The  temperature  rises  with  the  initial  sj'mptoms  and 
usually  reaches  its  maximum  on  the  third  to  the  fifth  day,  then  recedes 
by  lysis.  There  are  great  variations  in  the  intensity,  duration  and 
form  of  the  fever.  The  tendency  to  fall  by  lysis  is  almost  invariable; 
termination  by  crisis  is  exceptional  (p.  284,  Adolph  0.). 

Pulse. — ^The  pulse  is  relatively  rapid  (in  the  case  described,  168), 
but  regular.  The  rate  diminishes  with  the  drop  in  temperature.  Dur- 
ing convalescence,  as  after  other  severe  diseases  of  childhood,  there 
are  frequently  arrhythmia  and  bradycardia,  especially  during  sleep. 

Otherwise  no  Notable  Symptoms. — ^The  mind  is  clear  in  mild  and 
moderately  severe  cases  (the  group  of  cases  which  we  will  consider  in 


SCARLET  FEVER  273 

this  chapter) ;  there  is  some  lassitude.  During  the  first  few  nights 
sleep  is  somewhat  restless.  Every  marked  involvement  of  the  nervous 
system,  especially  stupor  and  convulsions,  indicates  a  severe  infection. 

The  larynx  and  lungs  are  not  involved  at  all  in  cases  of  moderate 
severity;  loss  of  voice  or  stenotic  sj'mptoms  point  rather  to  a  mixed 
infection  with  diphtheria. 

The  urine  during  the  first  two  weeks  shows  no  changes  except  for 
febrile  albuminuria.  We  have  never  seen  a  positive  nephritis  during 
the  first  days. 

Appetite  anil  indigestion  are  disturbed  only  as  long  as  the  fever 
lasts,  and  later  rapidly  become  normal.     Constipation  is  very  frequent. 

The  spleen  is  usually  not  palpable;  a  slight  enlargement  of  the 
liver  is  almost  always  present. 

Prognosis  in  Illustrative  Case. — ^Thc  prognosis  in  the  case  described 
cannot  be  definitely  stated  on  the  second  day  of  the  disease,  because 
the  sj'mptoms  arc  still  on  the  increase.  We  can  say,  though,  that  there 
is  no  immediate  danger  to  life,  and  that,  because  of  the  absence  of 
all  threatening  symptoms,  a  favorable  termination  is  probable.  The 
length  of  the  disease  is  even  less  determinable  on  account  of  the  possi- 
bility of  late  complications. 

Therapy. — ^^Ve  first  employ  the  following  therapeutic  measures 
(p.  311) :  Rest  in  bed,  soft  diet  and  no  meat  (milk  and  cereals),  a  moist 
compress  about  the  neck;  if  the  fever  rises  toward  evening,  a  wet  pack 
to  the  trunk.  Since  the  boy  is  old  enough  to  use  a  gargle,  we  prescribe 
a  1  per  cent,  solution  of  hydrogen  peroxide  for  this  purpose. 

DESCRIPTION  OF  THE  INDIVIDUAL  SYMPTOMS 

ST.\GE    OF    INCUBATION 

Duration,  3  to  4  days. — Most  authors  believe  that  the  time  between 
the  infection  and  the  appearance  of  the  initial  symptoms  of  scarlatina 
is  variable;  the  average  is  from  three  to  four  daj's.  The  exact  period 
of  incubation  is  difficult  to  determine,  because  the  disease  does  not 
always  occur  when  the  patient  is  exposed  to  the  infection. 

Joseph  B.,  six  years  old,  did  not  take  the  disease  until  fifty  days 
after  admission  into  the  scarlet  fever  ward,  where  he  had  been  brought 
as  the  result  of  an  incorrect  diagnosis. 

Only  the  minimal  period  can  be  positively  determined;  it  is  prob- 
ably less  than  twenty-four  hours  (Trousseau,  Soerensen). 

Johanna  F.,  two  years  old,  was  injured  by  a  machine  in  a  village,  in 
which  there  was  no  scarlet  fever.  Was  brought  to  the  city  in  the  morn- 
ing. At  noon  came  to  our  dispensary,  where  there  was  a  possibility  of 
infection.  Twenty-four  hours  later  scarlatinal  rash  appeared  without 
prodromal  symptoms. 

II— IS 


274  THE   DISEASES   OF   CHILDREN 

Anna  K.  was  isolated  at  her  grandmother's  home,  while  her  sister 
had  scarlet  fever.  Twenty-six  days  later  the  sister  returned  home 
from  the  hospital;  in  a  week  Anna  was  brought  from  her  grandmother's. 
She  came  home  at  eight  o'clock  in  the  morning,  was  cheerful  all  day;  at 
midnight  there  was  fever  and  restlessnesss;  in  the  morning  a  scarlatinal 
rash  was  noted.  The  incubation  in  this  case  was  sixteen  hours,  unless 
we  assume  it  to  have  been  thirty-four  days;  in  the  interim  there  had 
been  no  opportunity  for  infection. 

The  second  case  does  not  furnish  positive  evidence  because  in 
Vienna  an  infection  from  another  source  cannot  be  absolutely  excluded. 
The  first  example,  however,  is  excellent;  at  the  earliest  the  infection 
might  have  been  accjuircd  on  the  train,  about  thirty  hours  before  the 
appearance  of  the  rash.  In  practice  we  can  count  on  a  maximum  incu- 
bation of  eight  days;  we  must  keep  the  brothers  and  sisters  and  play- 
mates of  a  sick  and  isolated  child  under  observation  for  that  length  of 
time. 

TONSILLITIS    AND    .VSSOCIATED    SYMPTOMS 

Angina  Scarlatinosa. — Ordinarily  the  tonsils  are  the  site  of  infec- 
tion and  the  primary  focus  of  the  disease.  We  have  already  mentioned 
a  range  of  sevcrit)'  from  simple  infection  or  swelling  to  lacunar  deposits. 
The  follicular  form  disappears  in  many  cases  after  the  third  day,  swell- 
ing and  redness  alone  persisting  for  some  time. 

Membranous  Exudate.- — In  the  more  severe  cases,  after  the  second 
or  third  day,  the  follicular  patches  begin  to  spread  over  the  whole 
tonsils,  become  confluent,  and  pass  on  to  the  pillars  of  the  fauces,  the 
uvula,  and  more  rarely  the  posterior  wall  of  the  pharynx  (see  Plate  14). 
They  are  either  thin  and  white  or  dirty  and  yellowish  gray,  in  which 
event  they  differ  from  diphtheritic  membranes.  But  there  are  cases  in 
which  firm,  glistening,  white  membranes  develop,  which  cannot  be 
differentiated  from  diphtheria  macroscopically  either  bj"  their  color  or 
their  localization  (uvula,  arch  of  palate,  posterior  pharyngeal  wall). 

In  such  cases  only  microscopic  and  cultural  examination  can 
determine  difinitely  whether  the  membrane  is  due  to  streptococci  or 
to  diphtheria  bacilH.  If  such  an  examination  is  not  made,  it  is  safest 
to  inject  diphtheria  antitoxin,  because  true  nuxed  infections  of  scarlet 
fever  and  diphtheria  do  occur. 

S.  Gustav  (Fig.  48),  four  years  old;  eight  days  ago  sore  throat 
and  vomiting.  On  the  foUomng  day,  rash  and  high  temperature.  For 
four  days  a  pharyngeal  exudate  has  been  noted.  On  admission,  on  the 
ninth  day,  pigmented  remnants  of  the  rash,  beginning  desquamation, 
strawberry  tongue.  Pharynx  is  intensely  red  and  very  swollen.  The 
uvula  is  compressed  between  the  tonsils.  On  the  tonsils  and  uvula  is  a 
coalescent,  thick,  sharply  defined,  porcelain-white  membrane,  just  about 
to  separate  from  the  tonsil.     It  can  be  broken  up  easily,  in  the  micro- 


SCARLET  FEVER 


275 


scopic  specimen  (stained  by  Loffler's  methylene  blue)  are  found  only 
single  cocci  and  streptococci.  No  bacilli.  There  is  also  a  white  deposit 
on  the  posterior  pharyngeal  wall.     Speech  is  thick. 

On  the  tenth  day  the  deposits  are  creamy,  hght,  disappearing. 
Eleventh  day,  deposits  dehcate  wliite.  Thirteenth  day,  only  in  the 
crypts  of  the  tonsils  and  at  the  tip  of  the  uvula  are  there  white  patches, 
like  those  at  the  close  of  diphtheria.  Fifteenth  day,  pharyngeal  CEdema 
gone;   one  grayish  spot  in  a  crypt  of  the  right  tonsil. 


Fig.  48. 


6 


IL 


$     Posterior  pharyngeal  wall.     <S>     Posterior  palatine  arch.     ^     Membrane. 

a,  membrane  and  inflammation,  ninth  day  of  disease,     b.  eleventh  day — inflammation  less  intense,     c, 
thirteenth  day.     d,  seventeenth  day — small  patch  of  membrane. 

This  case  illustrates  a  favorable  course  in  a  pharyngeal  infection. 
It  is  more  common  in  scarlatina,  as  opposed  to  diphtheria,  that  when 
the  membrane  loosens,  the  superficial  layers  of  the  mucous  membrane 
also  separate,  so  that  the  pharyngeal  tissues,  especially  the  anterior 
pillars  of  the  fauces,  show  eroded,  scalloped  contours,  which  persist 
for  a  long  time,  and  are  gray  and  cloudy.  In  the  severest  cases  exten- 
sive necroses  of  the  tonsils  and  soft  palate  occur. 

Pharyngeal  Necroses. — Karl  St.  (Fig.  49),  four  years  old,  admitted 
on  the  seventh  day  of  the  disease.  Face  puffy,  purulent  nasal  discharge, 
l.ips  dry;  rhagades  at  the  angles  of  the  mouth.  Gums  bright  red 
and  covered  by  a  slimy,  wliite,  adherent  deposit.     Strawberry  tongue. 

Fig.  49. 


u,  membrane  on  tonsils,  uvula  and  anterior  palatine  arch,  eighth  day  of  disease,  b,  eleventh  day — 
fresh  membrane  on  rit;ht  palatine  arch.  c.  eighteenth  <lay — membrane  opposite  opening  in  anterior  pala- 
tine arch,  through  which  the  posterior  pharj-ngeal  wall  can  be  seen,  d,  twenty-fifth  da.v — hole  larger, 
marked  deformity  of  anterior  palatine  arch,  uvula  drawn  to  left  side. 


Pharynx  swollen;  bright  red  ;  on  the  uvula  and  pillars  of  the  fauces  are 
loose  gray  patches.    Smear  and  culture  show  streptococci. 

On  the  eleventh  day  of  the  disease,  otitis  media  appears  on  the 
right  side;  on  the  sixteenth  day  on  the  left  side;  on  the  twenty-third 
day,  nephritis.  Fever  continues  till  death  (on  the  thirtieth  day), 
ranging  from  39°  C.  (102.2°  F.)  to  40°  C.  (104°  F.).  In  spite  of  tliis,  the 
mind  remained  clear,  and  the  appetite  good  until  death. 


276  THE   DISEASES   OF   CHILDREN 

Progressive  Form. — The  pharyngeal  exudate  spread  and  did  not 
begin  to  ticparate  or  become  hniited  till  the  eighteenth  day.  AVhen  this 
occurred,  a  part  of  the  soft  palate  also  loosened;  the  arch  of  the  palate 
remained  for  a  while;  later  that  too  sloughed,  so  that  ultimately  almost 
the  entire  soft  palate  was  destroyed. 

But  such  occurrences  are  rare.  One  may  predict  that  even  with  a 
confluent  pharyngitis  the  patient  will  recover  in  the  second  or,  at  the 
latest,  in  the  third  week;  but  one  must  also  be  prepared  for  a  contin- 
uous fever,  until  resolution  has  taken  place. 

Protracted  Favorable  Course. — Emilie  G.  In  the  evening  emesis, 
fever,  exanthem.  In  the  morning,  fresh  generalized  scarlatinal  rash; 
cavity  of  the  mouth  bright  red;  thickly  coated  tongue.  Pharynx  very 
oedematous;  a  delicate  cloudiness  of  the  tonsils,  which  nearly  meet. 
Temperature  as  high  as  40°  C.  (104°  F.). 

Fourth  day:  In  the  tonsils  deep  indentations  covered  with  exudate. 

Thirteenth  day:  Tonsils  and  posterior  wall  of  pharynx  still  lightly 
coated;  fever  continuing  and  ranging  from  38°  C.  (100.4°  F.)  in  the 
morning  to  39°  C.  (102.2°  F.)  in  the  afternoon. 

Sixteenth  day:    Throat  clear. 

A  rare  anomaly  is  the  development  of  a  tonsillar  abscess. 

Tonsillar  Abscess. — Ludwig  P.,  13  years  old.  At  1  a.  m.,  sore 
throat  ;  at  noon,  vomiting  and  fever.  On  admission,  forty  hours  after 
onset,  scarlatinal  rash.  Pharyngeal  tissues  enormously  swollen ;  on  the 
right  side  the  anterior  pillar  of  the  fauces  protrudes  Uke  a  ball.  The 
uvula  is  oedematous,  erect;  there  are  dirty  yellow  patches  on  the  tonsils 
and  anterior  pillar.     Temperature  39°  C.  (102.2°  F.). 

Third  day:  Incision.  There  was  no  pus,  but  abundant  necrotic 
tags.  In  cultures  streptococci,  which  agglutinate  specifically  with 
Moser  serum.      Rapid  recovery. 

Extrabuccal  Primary  Lesion. — The  skin  also  may  be  the  seat  of 
the  primary  lesion,  though  only  after  a  break  in  its  continuity,  such  as 
cannot  be  demonstrated  when  the  primary  lesion  is  in  the  tonsils. 

Erwin  B.,  two  and  a  half  years  old.  Radical  operation  for  a  bilateral 
inguinal  hernia,  according  to  Bassini.  In  the  beginning,  normal  course. 
Four  days  later,  removal  of  the  skin  clamps.  On  the  following  day  a 
typical  scarlatina  rash  appears;  temperature  39.2°  C.  (102.4°  F.).  Buc- 
cal cavity  red;  tongue  hghtly  coated.  Tonsils  small  and  pale;  glands  at 
the  angle  of  the  jaw  not  palpable.  Normal  appearance  of  the  wound  on 
the  right  .side.  The  neighborhood  of  the  left  incision  is  diffusely  red- 
dened, swollen  and  very  tender.  A  few  of  the  sites  of  the  skin  clamps 
are  bright  red.  Inflammatory  changes  increased  on  the  following  day. 
Fluctuation;  incision;  20  c.c.  of  bloody  pus  escape.  In  the  smear  and 
culture  only  streptococci,  agglutinated  by  Moser  serum  in  dilution  of 
1:1000.    Third  day  of  the  disease:   Temperature,  40°  C.  (104°  F.);    the 


PLATE   1.5. 


SCARLET  FEVER  277 

edges  of  the  wound  are  infiltrated;  the  base  of  the  ulcer  shows  a  larda- 
ceous  deposit:  Strawberry  tongue;  the  wound  heals  very  slowly,  with 
the  formation  of  granulation  tissue.     Later  generahzed  desquamation. 

The  opinion  that  the  inguinal  ulcer  is  the  source  of  the  scarlatinal 
infection  is  based  on  the  appearance  of  the  wound,  wliich  presents  some 
resemblance  to  the  patches  of  a  scarlatinal  pharyngitis,  but  especially 
on  the  absence  of  every  pharyngeal  symptom.  The  diagnosis  of  scarlet 
fever  is  confirmed  by  the  characteristic  desquamation  following  the 
generalized  rash.  The  rash  and  strawberry  tongue,  as  we  have  men- 
tioned before,  belong  to  the  constitutional  symptoms  and  not  to  the 
primary  lesion.  They  therefore,  also  occur  as  a  result  of  an  extrabuccal 
infection. 

E.xtrabuccal  infections  are  not  very  rare.  We  have  seen  scarlet 
fever  begin  in  abrasions,  in  secondarily  infected  varicella  (Heubner), 
in  herpetic  vesicles,  and  pressure  vesicles  on  the  foot.  Naturally, 
any  wound  may  be  the  primary  focus.  Formerly,  when  scarlet  fever 
and  diphtheria  patients  were  not  strictly  separated,  tracheotomy 
wounds  were  frequently  the  infection  atrium  for  scarlatina. 

Cryptogenetic  Infections. — There  are  also  cases  in  which  it  is 
impossible  to  locate  the  primary  focus.  The  pharynx  is  simply  red- 
dened, the  cervical  lymph-nodes  are  not  palpable,  and  no  point  of 
entrance  can  be  found  in  the  skin.  Such  cryptogenetic  infections  are 
usually  cases  of  the  mildest  sort. 

Nose  and  Ears. — Affections  of  the  nose  are  very  frequent,  and,  as 
we  shall  presently  emphasize,  of  great  prognostic  significance.  In  mild 
cases  there  is  simply  a  sHght  serous  discharge;  in  more  severe  forms 
the  nose  is  occluded,  the  orifices  are  excoriated  and  swollen,  the  dis- 
charge is  thick,  profuse  and  mucopurulent.  The  latter  depends  upon 
a  severe  inflammatory  change  in  the  mucous  membrane  of  the  nose, 
which  may  progress  to  necrosis  and  membranous  exfohation  of  the 
superficial   tissues  (Uffenheimer). 

The  nasal  involvement  usually  sets  in  on  the  third  or  fourth  day 
of  the  disease.  It  may  be  very  protracted  and  often  delays  deferves- 
cence. The  children  are  very  much  annoyed,  especially  during  sleeji, 
by  the  obstruction  to  nasal  respiration  and  the  dryness  of  the  mouth 
resulting  from  it. 

Otitis — By  way  of  the  Eustaclaian  tube,  involvement  of  the  nose 
and  nasopharynx  frequently  leads  to  otitis  media,  wliich  often  ends  in 
chronic  otorrhoea.  Otitis  media  is  a  favorite  comphcation  of  scarlet 
fever.     It  may  also  occur  without  disease  of  the  nose. 

Joseph  B.,  nine  years  old.  Second  day  of  the  disease:  Typical  scarlet 
rash.  On  the  tonsils  folhcular  patches ;  nose  dry ;  ear  drums  [lale. 
Sixth  day:  Temperature  38.2°  C.  (100.8°  F.);  pharynx  clean;  right 
tympanic    membrane   deep   red,   left    slightly   injected ;    no   subjective 


278  THE   DISEASES   OF   CHILDREN 

symptoms.     Compresses   of   acetate   of   aluminium    (1   per   cent.)    and 
instillation  of  carbolic  acid  in  glycerin. 

On  the  following  days  increase  in  inflammatory  findings;  tem- 
perature remaining  about  38°  C.  (100.4°  F.).  After  the  eighth  day, 
inflammation  and  fever  decrease. 

Fourteenth  day  :  Tympanic  membranes  pale ;  no  perforation 
occurred. 

Otitis  does  not  always  terminate  so  favorably;  but  even  when  it 
results  in  spontaneous  perforation,  pain  may  he  absent.  Small  cliildren 
also  may  localize  the  pains  incorrectly.  Tenderness  of  the  opening  of 
the  outer  ear  may  furnish  a  clue  to  the  existence  of  an  otitis.  It  can  be 
excluded  only  by  inspection  of  the  drum,  which  can  be  done  better  in 
children  without  a  speculum,  because  the  introduction  of  a  speculum 
causes  pain  and  restlessness.  In  the  absence  of  premonitory  symptoms, 
one  is  often  surprised  by  the  onset  of  an  otorrhoea. 

Otto  S.,  two  years  old.  Second  day  of  disease:  Slight  mucous  dis- 
charge from  nose.  Up  to  sixth  day  of  disease,  gradual  rise  of  tempera- 
ture to  40.2°  C.   (104.4°  F.). 

Fifth  day:  Right  sided  otorrha?a  begins  without  subjective  symp- 
toms.    The  left  tympanum  is  inspected  and  found  to  be  reddened. 

Sixth  day:  Injection  increased,  bulging;  paracentesis;  drop  in  tem- 
perature to  37.8°  C.  (100°  F.).  Temperature  falls  by  lysis.  Discharge 
still  present  from  ears,  however,  at  time  of  dismissal  on  fifty-sixth  day. 

Termination  in  Chronic  Otorrhcea.  Mastoiditis. — Chronic  otor- 
rhoea may  continue  for  years  and  result  in  impairment  of  hearing.  The 
condition  becomes  more  grave,  when  the  inflammatory  process  extends 
to  the  mastoid  bone.  One  must  think  of  this  complication,  when  the 
region  behind  the  car  is  strikingly  sensitive.  If,  in  spite  of  cold  com- 
presses, the  symptoms  increase,  and  redness  and  swelling  appear,  the 
diagnosis  may  be  made  with  certainty.  It  is  essential  to  avoid  con- 
founding this  condition  with  suppurative  lymphadenitis  of  the  region. 

Franz  0.,  seven  years  old.  Mild  scarlet  fever.  On  the  thirteenth 
day  of  illness,  pains  in  the  right  ear,  ear  drums  pale  and  thickened.  On 
the  sixteenth  day  spontaneous  perforation  of  right  tympanum,  abun- 
dant mucopurulent  discharge.  At  the  same  time  a  painful  swelling 
over  right  mastoid  process.  The  skin  red  and  oedematous  over  it.  In 
the  afternoon  rapid  rise  of  temperature  to  40.2°  C.  (104.4°  F.). 

Seventeenth  day:  Application  of  leeches;  pain  less,  otherwise  no 
changes. 

Eighteenth  day:  Trephining;  thick  pus  in  the  antrum.  Suppura- 
tive periostitis.     Complete  recovery. 

Pauline  E.,  nine  years  old.  On  admission,  on  fourth  day,  severe 
infectious  changes  of  throat  and  nose ;  dacryocystitis.  On  the  left 
side,  marked  injection  and  bulging  of  tympanum;    spontaneous  perfor- 


SCARLET  FEVER  279 

ation  in  left  posterior  quadrant;  right  tympanum  normal.  Secretions 
from  left  ear  increase;   markeil  tenderness  of  external  meatus. 

Eleventh  day:   Paresis  of  left  facial  nerve. 

Twelfth  day:   Tip  of  mastoid  process  tender. 

Fourteenth  day:    Right  tympanum  red  and  bulging;    paracentesis. 

Sixteenth  day:   Left  facial  paralysis.    Secretion  from  ear  decreasing. 

Twentieth  day:  Beginning  power  of  motion  in  the  region  of  supply 
of  the  frontal  branches  of  the  facial  nerve.     Fetid  discharge. 

Two  months  later,  radical  operation:  Abscess  of  the  left  mastoid 
process  ;   recovery. 

The  disease  of  the  mastoid  process  may  extend  to  the  meninges 
and  rapidly  lead  to  death. 

Franz  K.,  seventeen  months  old.  Moderately  severe  scarlet  fever 
with  nasal  involvement.  Till  the  nineteenth  day,  remittent  tempera- 
ture, produced  by  left-sided  otitis  media.  On  the  twenty-third  da)% 
sudden  convulsions;  one  and  one-half  hours  later,  death.  At  autopsy, 
a  brain  abscess  as  large  as  a  hazel-nut  was  found  in  the  left  frontal 
lobe,  originating  from  mastoid  process.  Such  an  abscess  may  exist  for 
a  considerable  period  without  symptoms. 

Wilhclm  Sch.  Had  scarlet  fever  at  three  years.  Otitis.  A  year 
and  a  half  later,  he  suddenly  became  ill  with  symptoms  of  brain  tumor. 
Several  days  later,  general  convulsions;  coma.  On  lumbar  puncture, 
a  thick,  purulent  fluid  escaped.  The  diagnosis  of  brain  abscess  with 
rupture  into  the  ventricle,  following  otitis,  was  confirmed  by  autopsy. 

Lymph-nodes. — A  moderate  swelling  of  the  neighboring  lymph- 
nodes  is  a  regular  accompaniment  of  the  primary  lesion.  In  cxtra- 
buccal  infections  the  corresponding  lymph-nodes  are  affected ;  those 
at  the  angle  of  the  jaw  remain  free. 

There  is  no  fixed  proportion  between  the  intensity  of  the  pharyn- 
geal process  and  the  swelling  of  the  nodes.  In  spite  of  a  severe  pharyn- 
gitis, the  nodes  may  be  scarcely  palpable;  they  may  suppurate,  when 
the  involvement  of  the  throat  is  insignificant. 

Leopold  H.,  three  years  old.  On  the  fourth  day  of  the  disease, 
moderate  redness  of  the  pharynx;  no  patches  on  the  tonsils.  At  the 
angle  of  the  left  jaw,  a  lymph-node  as  large  as  a  walnut.  The  induration 
of  the  node  increases  for  the  next  few  days,  with  continuous  fever.  On 
the  eleventh  day,  the  node  is  as  large  as  a  goose  egg.  On  the  fourteenth 
day,  there  is  diffuse  and  tender  infiltration  of  the  tissues.  On  the  fol- 
lowing day,  fluctation. 

Sixteenth  day:  Incision  ;  escape  of  abundant  greenish  pus,  which 
contains  streptococci.  Later  rapid  drop  in  temperature;  gradual  closure 
of  wound. 

Abscess  Formation. — In  the  severest  cases  of  the  infectious  type  the 
entire  floor  of  the  mouth  and  all  the  deep  lymph-nodes  of  the  neck  maj' 


280  THE   DISEASES   OF   CHILDREN 

become  involved  in  the  inflammatory  process;-  a  firm  and  higii  grade 
infiltration  results  (angina  Ludovici),  which  may  produce  asphyxiation 
by  compression  of  the  trachea.  Such  cases  are  doomed;  tracheotomy 
delays  death  only  a  few  hours  or  days. 

Walter  N.,  thirteen  months  old.  On  the  fourth  day  of  illness  a 
scarlatinal  rash,  partly  small  macular,  partly  papular.  Temperature 
41.2°  C.  (106.2°  F.).  Pulse  180;  respirations,  64;  convulsions.  Pharynx 
swollen;  dirty  exudate  on  the  tonsils;  severe  rhinitis.  At  the  right 
angle  of  the  jaw,  a  lymph-node  as  large  as  a  walnut;  on  the  left  side 
diffuse  infiltration.  Constitutional  symptoms  diminish  after  adminis- 
tration of  Moser  serum.  Temperature  drops  to  38.8°  C.  (101.8°  F.); 
infiltration  about  the  neck  increases. 

Ninth  day:  Board-hke  infiltration  of  the  entire  cervical  region; 
Temperature  about  40°  C.  (104°  F.). 

Eleventh  day:  Pharynx  markedly  narrowed  by  swelling.  Exudate 
on  tonsils  and  pillars  increased.  Nose  occluded.  The  infiltrate  about 
the  neck  is  beginning  to  soften. 

Twelfth  day:   Incision;   thin  pus  with  necrotic  tags;   drainage. 

Thirteenth'  day:  The  incision  is  wide  open;  no  secretion;  sur- 
rounding tissue  shows  board-like  infiltration.     Purulent  bronchitis. 

Fourteenth  day:  Increasing  induration  of  tissues  of  neck;  death 
by  asphyxia. 

In  other  cases,  transmission  of  the  suppurative  process  may  ter- 
minate in  mediastinitis.  Death  may  also  result  from  erosion  of  the 
carotid  artery.  But  these  are  only  rarities  which  are  to  be  expected 
only  in  the  severest  forms  of  the  disease. 

CONSTITUTION.\L    SYMPTOMS 

Initial  Symptoms. — At  the  very  onset  of  scarlet  fever,  aside  from 
the  sore  throat,  caused  by  the  primary  lesion,  one  symptom  is  frequent 
and  pronounced — the  initial  vomiting. 

The  rash  usually  appears  a  few  hours  after  the  vomiting;  the 
temperature  rises  rapidly  from  the  very  beginning:  it  attains  its 
maximum  during  the  development  of  the  later  scarlatinal  symptoms. 
Exceptionally  the  rash  appears  before  the  vomiting. 

W.  Anna,  three  years  old.  Infected  in  hospital.  At  5  a.  m.  no 
fever.  Temperature,  36.9°  C.  (98.4°  F.).  Sleepy  at  breakfast.  Tem- 
perature, 37.3°  C.  (99.1°  F.).  At  10  a.  m.,  indistinct  pale  red,  small 
macular  rash  on  chest  and  abdomen.  Mucous  membrane  of  mouth 
slightly  reddened;  that  of  pharynx  more  distinctly  so,  and  moderately 
swollen.  At  7  p.  m.,  temperature  39.9°  C.  (103.8°  F.);  pulse  164.  Rash 
over  entire  body.  At  11:30  p.  m.  vomited  once.  Mild  course.  In  very 
mild  cases  the  rash  may  appear  without  any  other  symptoms. 

Karl  H.,  admitted  by  mistake  to  scarlet  fever  ward  (hypersemia 


SCARLET  FEVER  281 

due  to  crying).  Four  days  later,  without  preceding  fever,  a  typical 
delicate  rose-red  rash  appeared  on  the  face  and  extremities.  Thin 
superficial  deposit  on  the  tonsils;  pharj'nx  reddened,  especially  about 
the  right  pillar  of  fauces.  Maximum  temperature,  38°  C.  (100.4°  F.),  on 
the  fourth  day  of  disease. 

Since  the  rash  may  appear  simultaneously  -with  or  before  the  other 
initial  symptoms  or  without  them,  the  separation  of  a  distinct  pro- 
dromal stage  is  not  indicated. 

In  mild  cases,  vomiting  usually  is  not  repeated.  Persistent  nausea 
for  one  or  two  days  is  unfavorable  from  a  prognostic  point  of  \'iew, 
since  it  usually  indicates  severe  toxic  forms  of  the  disease. 

Herpes. — Herpes  is  a  rare  initial  symptom.  We  have  seen  it  in 
only  fom-  cases,  two  of  whom  were  members  of  the  same  family. 

Headache,  lassitude  and  general  malaise  are  often  among  the  first 
symptoms.  The  appetite  is  poor  ;  there  is  usually  constipation ;  in 
severe  toxic  forms,  on  the  other  hand,  there  are  initial,  profuse,  green, 
maladorous,  diarrhoeal  stools. 

The  Rash. — The  rash  of  typical  scarlet  fever  cases,  as  we  have 
mentioned  in  the  description  of  the  general  chnical  course,  is  very 
easily  recognized.  The  plates,  in  three  color  type,  drawn  from  wax 
casts,  show  such  types  of  exanthemata  better  than  we  can  describe  them 
in  words  (Plates  15  and  16). 

In  scarlet  fever  there  appear,  besides  the  typical  bright  red,  fine, 
macular  hj'pertemias,  two  forms  of  exanthemata.  These  do  not  occur 
independenth^,  but  superimposed  upon  the  other:  (1)  swelling  of  the 
follicles,  which  may  progress  to  pustulation;  and  (2)  the  morbilliform 
type,  characterized  by  large,  brownish  red,  shghth'  elevated  papules. 

Heinrich  K.,  twelve  years  old. 

First  day:   Fever,  sore  throat,  emesis,  diarrhoea. 

Second  day:    Rash. 

Third  day:  (Day  of  admission).  Very  small,  bright  red  macules  over 
entire  body.  On  the  flexor  surface  of  the  extremities,  on  the  external 
surface  of  the  shoulders  and  neck,  numerous  pinhead-sized  nodules,  corre- 
sponding to  the  folHcles,  which  on  the  back  remind  one  of  goose-flesh. 

Fourth  day:  Increase  of  foUicular  swelhng  on  the  backs  of  hands 
and  feet,  and  on  the  extensor  surface  of  fingers  and  toes. 

Fifth  day:  The  fine  macular  rash  has  become  paler,  the  contents  of 
many  of  the  folUcles  hquefied,  appear  wliitish. 

Sixth  day:  New  groups  of  vesicles  about  knee-joints;  the  older 
ones  on  the  upper  extremities  beginning  to  desiccate. 

Seventh  day:  Vesicles  all  dried. 

Eighth  day:   Punctiform  desquamation  at  tips  of  follicles. 

Seventeenth  day:  Desquamation,  which  had  been  sUght,  assumes 
a  lamellar  type,  especially  about  the  feet. 


282  THE   DISEASES   OF   CHILDREN 

This  case  illustrates  the  fact  that  the  formation  of  vesicles  is  only 
a  later  stage  in  the  course  of  a  folhculitis. 

The  intensity  of  the  fine  macular  and  folhcular  rashes  is  of  httle 
prognostic  significance.  On  the  other  hand,  asymmetrical  distribution, 
mixture  with  morbilliform  lesions  (double  exanthema),  and  cyanotic 
and  dirty  shades  of  color  have  an  unfavorable  significance,  because 
these  forms  belong  chiefly  to  the  severe  infectious  or  toxic  types  of 
scarlet  fever.  But  an  unfavorable  prognosis  should  not  be  based  solely 
upon  the  rash;   the  other  symptoms  must  also  be  considered. 

Karl  L.,  three  years  old. 

First  day:    p.  m.,  vomiting,  evening  fever. 

Second  day:  Rash,  temperature  38.5°  C.  (101.3°  F.);  evening, 
41°  C.  (105.8°  F.):    restlessness. 

Third  day:  (On  admission).  Universal  dense,  small  macular, 
bright  red  exanthem,  confluent  on  back.  Face  shghtly  puffy,  cheeks 
red,  pale  circumoral  triangle  (the  triangle  which  gives  a  yellow  or  pale 
effect,  and  is  drawn  from  the  root  of  the  nose  to  the  angles  of  the  mouth, 
and  thence  to  the  chin).  On  the  inner  surface  of  the  arms,  on  the  backs 
of  hands  and  on  the  anterior  surface  of  the  tliighs,  larger,  dark  brownish 
red,  shghtly  elevated  efflorescences.  Feet  shghtly  cyanotic;  sore  throat, 
coryza,  lassitude. 

Fourth  day:  Rash  brownish  and  cyanotic,  in  some  places  hsemor- 
rhagic  spots:  Severe  constitutional  symptoms,  nausea,  prostration. 
Death  on  seventh  day. 

Hmmorrhagic  types  of  the  rash  are  said  to  occur;  we  have  never 
seen  them.  Sometimes  there  may  be  very  small  points  of  hgemorrhage 
in  the  folds  of  the  skin  (axilla,  elbow);  they  can  be  explained  on  me- 
chanical grounds.  They  are  also  found  on  the  back,  where  the  bed- 
clothing  Ues  in  folds,  and  thus  produces  stronger  pressure.  We  believe 
these  hsemorrhages  to  be  e'vidence  of  shght  changes  in  the  smallest 
blood  vessels,  the  result  of  increased  vulnerabihty.  Tliis  appearance 
can  easily  be  produced  artificially  by  picking  up  and  squeezing  a  fold 
of  skin  between  both  thumbs  and  index  fingers.  The  skin  of  healthy 
children  must  be  pinched  very  hard  to  produce  a  ha?morrhagc.  But 
every  hypera?mic  skin  will  give  rise  to  such  hiemorrhages,  even  if  less 
easily  than  in  scarlet  fever.  Still  the  difference  is  not  sufficiently  pro- 
nounced to  make  this  .symptom  valuable  in  the  differential  diagnosis. 

Anna  L.,  twelve  years  old.  Very  mild  attack,  with  fever  lasting  only 
three  days.  On  third  day  of  illness  showed  punctate  hemorrhages  in 
many  places,  which  may  also  be  produced  easily  by  pressure  on  the  skin. 

Scarlatina  Withovt  Rash. — The  rash  not  only  varies  greatly  in 
color,  intensity  and  form,  but  may  be  entirely  absent. 

Juhe  Z.,  twelve  years  old.  Admitted  at  the  same  time  as  two  others 
in  the  family,   who  have  a  typical  scarlet  fever.     Patient  became  ill 


SCARLET  FEVER 


283 


during  the  night  ^\•ith  sore  throat  and  fever.  The  pillars  of  the  fauces 
and  tonsils  are  bright  red;  on  the  latter  are  white  spots  as  large  as 
flaxseeds.  Uvula  intensely  red,  shghtly  (i>dematous.  Tongue  normal. 
Temperature  39.2°  C.  (102.5°  F.).  None  of  the  typical  complications 
occurred  in  this  case,  so  that  the  diagnosis  is  based  only  upon  the  illness 
of  the  other  members  of  the  family. 

Scarlatinal  pharyngitis  without  rash  is  of  importance,  because  such 
cases  are  usually  not  recognized  as  scarlet  fever  and  are  therefore 
especially  hable  to  spread  the  infection. 


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Marie  0.,  four  years  old.  Typical  pharyngeal  scarlatina,  wthout 
rash.     She  infected  three  of  her  family  and  herself  fell  ill  with  a  nephritis. 

Still,  the  absence  of  a  rash  is  not  common.  In  the  liistory  of 
patients  with  nephritis  it  is  often  stated  that  sore  throat,  but  no  rash, 
had  preceded  it.  In  many  of  these  cases  probably  the  rash  had  been 
overlooked  on  account  of  its  transitory  character. 

Isolated  cases  of  scarlatinal  pharyngitis  are  very  difficult  to  recog- 
nize; they  may  be  conjectured  on  account  of  the  inten.se  redness  of 
the  soft  palate,  but  it  is  better  to  isolate  the  suspicious  cases  and  not  to 
admit  them  to  a  scarlet  fever  ward. 

Marie  E.,  two  and  a  half  years  old.     Showed  intense  redness  and 


284 


THE   DISEASES   OF   CHILDREN 


Fig.  si. 


swelling  of  the  mucous  membrane  of  the  mouth  and  pharynx.  The 
tongue  was  strawberry-like;  the  lacunar  exudates  grayish,  containing 
streptococci.  A  week  after  admission  to  the  scarlet  fever  ward  she 
acquired  a  typical,  severe  scarlatina. 

The  pharyngeal  involvement,  therefore,  had  not  been  the  expression 
of  a  scarlet  fever,  in  spite  of  its  intense  redness  and  the  strawberry  tongue. 
Fever. — The  condition  of  the  temperature  is  not,  as  in  diphtheria, 
of  subordinate  significance,  but  is  intimately  dependent  upon  all  phases 
of  the  disease.  We  have  seen  that  most  of  the  complications,  too,  are 
introduced  and  accompanied  by  an  increase  of  fever.  Therefore,  con- 
tinuous   observations  of   temperatures  are    absolutely  necessary,   well 

into  convalescence. 
In  our  introduction 
we  have  described 
the  normal  course 
of  the  fever:  Rise 
of  temperature  with 
the  appearance  of 
the  rash  and  defer- 
vescence by  lysis, 
beginning  on  the 
third  to  the  fifth 
day  (Fig.  50).  Ex- 
ceptions from  this 
type  are  frequent  in 
complicated  cases, 
but  also  occur  in  un- 
complicated forms. 
Atypical  Course. 
— Fall  of  tempera- 
ture by  crisis  on 
the  second  or  third 
day  of  the  disease 
occurs  rarely  in 
otherwise  mild  cases,  and  only  very  exceptionally  in  such  cases  as 
present  other  signs  of  a  Severe  infection. 

Fall  by  Crisis. — Adolph  0.  (Fig.  51),  five  years  old.  In  the 
morning,  vomiting,  fever.  Afternoon,  temperature  38.6°  C.  (101.5°  F.); 
rash.  On  the  following  morning,  temperature,  40°  C.  (104°  F.);  pulse 
150.  Small  macular  rash  of  varying  color.  Cyanosis  of  the  feet.  Sev- 
eral times  the  extremities  became  cold;  there  was  lassitude.  Prognosis 
III  (doubtful)  was  given.  At  noon  temperature  rises  to  40.2°  C.  (104.4° 
F.);  then  a  spontaneous  drop  to  37.5°  C.  (99.5°  F.)  on  the  following 
morning.    Disappearance  of  all  symptoms. 


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SCARLET  FEVER 


285 


We  have  never  seen  temperature  curves,  such  as  occur  in  pneu- 
monia, in  which,  after  several  days  of  continuous  fever,  a  spontaneous 
crisis  appears. 

Irregular  rises  and  falls  are  frequent,  during  the  first  week,  often 
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cause  have  been  known  since  Fiirbinger  as  "after-fever."  In  most 
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Therefore  one  should  employ  the  term  "after-fever"  only  when  an 
examination  of  all  of  the  organs  is  negative. 


286 


THE   DISEASES   OF   CHILDREN 


Albert  Sell.  (Fig.  53),  six  years  old.  The  representation  of  all  of 
the  various  symptoms  on  the  chart  gives  us  an  idea  of  the  different 
phases  of  the  disease.  Fever  continues  to  the  twenty-second  day  and 
results  from  various  causes,  finally,  from  a  mild  nephritis.  The  albu- 
minuria and  interference  with  elimination  of  fluitl  are  graphically 
represented  in  the  drawing. 

The  height  of  the  fever  is  a  valuable  indication  of  the  severity  of 
the  primary  infection,  because  cases  with  fever  less  than  38°  C.  ( 100.4° 
F.)  on  the  second  or  third  day  almost  invariably  are  mild,  while  those 
as  high  as  40.5°  C.   (105°  F.)  must  always  be  considered  serious.     A 

Fig.  53. 


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',  "^^."'^r 

;■  ■  rr^ 

Continued  fever  in  scarlet  fever. 

temperature  of  from  38°  C.  (100.4°  F.)  to  40°  C.  (104°  F.),  is  by  itself 
of  little  prognostic  significance. 

In  the  ver}'^  mildest  forms,  fever  may  be  entirely  absent. 

Rosalie  P.,  four  years  old.  Infection  in  the  hospital,  observed 
from  inception.  Ran  a  course  with  typical  rash  and  desquamation, 
without  fever  or  rapid  pulse. 

Heart. — The  heart  is  distinctlj^  involved  in  every  moderately 
severe  case  of  scarlet  fever.  The  earliest  evidence  is  a  marked  tachy- 
cardia, which  is  often  much  greater  than  would  be  expected  from  the 
temperature.  Pulse  rates  of  150-160  are  not  unusual  nor  necessarily 
of  dangerous  significance. 

Otto  T.,  four    and   a   half    years    old.      During    observation    for 


PLATE  16. 


W    3 


w  a 


SCARLET  FEVER  287 

seventeen  days  before  the  onset  of  the  ilhicss,  pulse  rate  ranged  from  88 
to  114.    On  the  day  of  onset,  temperature  30.8°  C.  (98.2°  F.);  pulse  90. 

First  clay:  Right  posterior  pillar  of  fauces  markedlj'  reddened. 
Scarlatinal  rash  without  emesis. 

Second  day:  Temperature,  .38°  C.  (100.4°  F.)  to  39.2°  C.  (102.5° 
F.).  Pulse  1.5U.  Rash  has  advanced  to  the  inner  surfaces  of  the  ex- 
tremities. 

Third  day:  Temperature,  39.2°  C.  (102.5°  F.).  Puke  200.  After 
this,  reduction  in  the  pulse  rate,  which  fluctuates  between  114  and  138, 
and  does  not  return  to  96  till  the  twenty-third  day.  Normal  tempera- 
ture from  the  tenth  day  on. 

Pronounced  dilatation  of  the  heart  is  rarely  demonstrable. 

Stefan  V.,  admitted  on  thirtl  day  of  illness.  Confluent  cyanotic 
discoloration  of  cheeks.  On  the  back,  a  duskj-  red,  dense  rash.  On  the 
extremities  faint  efflorescences.  Hands  are  diffusely  brownish  red; 
scattered  irregularh'  about  the  body  are  raised  spots,  varying  from  pin- 
head  to  spht  pea  size,  in  addition  to  the  small  macular  rash.  There 
is  grinding  of  the  teeth;  and  coma.  Ape.x,  1§  cm.  outside  of  the  nipple 
line,  in  fourth  interspace.  Pulse  small  and  weak  (180),  regular.  Stools 
liquid.  Intense  pharyngitis.  Temperature,  40.3°  C.  (105°  F.).  Two 
hundred  c.c.  scarlet  fever  serum  (Moser)  injected.  On  the  follow- 
ing day,  mind  clear;  rash  paler,  cyanosis  disappeared.  Temperature 
38.2°  C.  (100.8°  F.);  pulse  144.  Apex  beat  inside  of  the  nipple  hne. 
Recovery. 

Inflammatory  changes  of  the  myocardium  and  endocardium  are 
difficult  of  clinical  demonstration.  Post-mortem  examinations  show 
that  the}'  are  frequently  present  in  fatal  cases. 

Endocarditis. — We  have  seen  the  onset  of  an  endocarditis  as  early 
as  the  ninth  da}'    of  illness. 

Hermann  M.,  six  years  old.  Moderately  severe  case.  Deferves- 
cence by  lysis  to  38°  C.  (100.4°  F.).  From  the  ninth  day  on,  step-like 
rise  in  temperature  to  the  twelfth  day:  40.6°  C.  (105.8°  F.).  Then  a 
gradual  fall  until  the  nineteenth  day. 

Fifth  day,  the  heart  action  became  irregular  antl  slow,  70  to  90. 

Ninth  day,  systolic  murmur  at  apex  and  in  pulmonic  area. 

Next  day,  murmur  was  more  distinct  and  palpable. 

Fourteenth  day,  dilatation  of  heart  to  the  left.  Systolic  murmur 
becomes  fainter  after  the  thirtieth  day,  and  on  the  fortieth  is  no  longer 
audible.      Heart  dulness  is  again  normal;   action  not  arrhythmic* 

In  this  case,  as  far  as  could  be  clinically  determined,  there  was 
recovery  from  the  endocarditis  with  perfect  function  of  the  valves.     In 


*I  have  not  infrequently  noticed  the  occurrence  of  cardiac  murmurs  during  the  first  two  weeks  of  the 
disease,  together  with  otlier  chanees.  wliich  proliably  indicate  myocardial  dcReneration.  Tlje  murnuir  has 
usually  been  interpreted  as  accidental  in  character  or  due  to  dilatation,  in  consequence  of  luyoc^rditis, 
especially  if  it  disappears  completely  in  a  short  time. — I.  ,\.  ,\. 


288  THE   DISEASES   OF   CHILDREN 

other  cases,  a  valvular  defect  persists.  It  is  well  known  that  next  to 
rheumatism,  scarlet  fever  is  the  most  frequent  cause  of  early  acquired 
valvular  lesions. 

Systolic  Mumurs.— Systolic  murmurs  without  dilatation  freiiuently 
occur  in  the  course  of  scarlet  fever.  Usually  they  disappear  rapidly. 
We  do  not  believe  that  one  is  justified  in  attributing  these  murmurs 
to  inflammatory  changes  of  the  •endocardium. 

Sudden  heart  failure  (Plerztod)  during  convalescence,  such  as  occurs 
in  diphtheria,  does  not  take  place  in  scarlet  fever. 

[(That  sudden  death  from  cardiac  failure  may  occur  was  exempli- 
fied by  a  case  which  occurred  in  my  own  practice  during  the  last  winter. 
A  child,  of  feeble  constitution,  six  years  of  age,  had  gone  through  a 
moderately  severe  scarlet  fever,  with  diffuse  rash,  marked  angina, 
rapid  pulse,  and  slight  nephritis.  The  child  had  been  fed  somewhat 
liberally  after  the  fifteenth  day  of  the  illness  and  seemed  to  be  conva- 
lescing. On  the  twentieth  day  it  was  sitting  up  in  bed,  playing  and 
chatting,  when  suddenly  it  became  restless,  pallid ;  it  assumed  the 
recumbent  position;  the  heart's  action  became  extremely  rapid;  the 
labored  breathing  and  cyanosis,  together  with  the  presence  of  numerous 
moist  rales,  indicated  the  occurrence  of  a  pulmonary  oedema.  Death 
occurred  in  a  few  hours. — I.  A.  A.)  (See  also  Romberg,  in  Deutsche 
Arch.,  f.  klin.  Med.,  Vol.  40,  and  Heubner,  Lchrbuch  f.  Kinderheilkunde.)] 

Scarlatinal  Rheumatism. — It  is  impossible  to  say  whether  the 
transitory  rheumatoid  affections  of  primary  scarlet  fever  should  be 
grouped  with  the  toxic  or  with  the  infectious  symptoms.  They  usually 
appear  at  the  end  of  the  first  week.  They  are  characterized  by  their 
early  involvement  of  different  joints,  especially  of  hands  and  feet,  and 
by  their  benign  and  rapid  course.  Older  children  of  their  own  accord 
complain  of  severe  pain  in  the  affected  joints.  In  younger  children 
pain  is  elicited  by  motion. 

Afebrile  Rheumatism.  —  Stefan  B.,  thirteen  years  old.  Mild 
scarlatina. 

Fifth   day:     In   afternoon,    tenderness   in    both    wrists. 

Seventh  day:  Spontaneous  pain  in  the  phalangeal  joints;  skin 
over  them  and  the  dorsum  of  the  hand  and  wrists  slightly  swollen, 
red,  and  hot. 

Eighth  day:  All  symptoms  have  subsided.  In  this  case  fever 
was  absent;    usually  fever  is  protracted  by  the  joint  affection. 

Rheumatism  v^ith  Fever. — Franz  H.,  seven  years  old.  On  the 
fifth  day  of  a  mild  scarlatina,  painful  swelling  of  the  right  wrist.  On 
the  seventh  day,  same  on  the  left  side. 

Eighth   day:    Left  phalangeal  joints  swollen. 

Ninth  day:  Head  to  left  side;  pains  in  the  left  side  of  the 
necK;,   which    continued    till   the   eleventh   day.     Pain  in   wrist-joints 


SCARLET  FEVER  289 

disappear?;  on  the  tenth  day.  During  arthritis,  fever  as  high  as  39.1°  C. 
(102.4°  F.I. 

It  i.<  important  to  know  that  these  joint  affections  always  ter- 
minate favorably  and  are  different  from  suppurative  arthritides.  The 
latter  occur  only  in  the  severest  infectious  cases. 

We  have  seen  this  complication  only  once,  in  a  girl  nine  years 
old,  Hermine  Z.  On  twelfth  day  of  the  disease  the  left  elbow,  on  the 
following  day  the  right  wrist,  began  to  suppurate.  Death  on  the  four- 
teenth day.    Pya'mia. 

Differential  Diagnosis. — We  refer  for  the  diagnosis  to  the  descrip- 
tion of  the  clinical  symptoms  (see  p.  268). 

The  following  diseases  may  call  for  differential  diagnosis  on  ac- 
count of  the  rash: 

1.  Reddening  of  the  skjn  in  febrile  diseases. 

2.  Hyperemia  due  to  crying. 

3.  Sudamina  and  eczema  of  young  children,  especially  during 
first  year  of  life. 

4.  Irritation  of  the  skin  by  moist  or  oily  compresses. 

5-7.  Infectious  exanthemata  (measles,  rubella  and  erythema 
infectiosum). 

8.  Drug  rashes. 

9.  Scarlatiniform  rash  resulting  from  injection  of  sera. 

1.  Febrile  Erythema. — ^The  erythema  of  the  skin,  resulting  from 
fevers,  does  not  show  a  small  macular  configuration.  It  is  important 
to  examine  the  skin  on  the  inner  surface  of  the  thigh. 

2.  Exanthem  from  Crying. — ^The  most  characteristic  sign  of  hyper- 
semia  due  to  crying  is  its  complete  disappearance  when  the  child  is 
quieted.  The  hypersemia  resembles  blushing;  both  affect  chiefly  the 
upper  part  of  the  body,  and  the  extremities  not  at  all.  Confusion  with 
incipient  scarlet  fever  is  possible,  especially  if  sore  throat  is  present. 

We  have  observeil  a  twelve-year-old  girl,  Marie  Z.,  brought  up  in 
an  orphan  asylum,  who  was  sent  to  the  hospital  three  times  with  the 
diagnosis  of  scarlet  fever.  In  each  instance,  there  was  only  a  lacunar, 
febrile  tonsillitis.  Every  time  the  body  of  the  child  was  examined  by 
the  physician,  a  rose-red  color  appeared  on  the  trunk.  The  error  oc- 
curred the  more  easily,  because  on  closer  examination  the  rash  was 
found  to  be  composed  of  minute  macules;  the  cheeks  were  flushed; 
the  face  about  the  mouth  was  pale,  and  the  pharj'nx  bright  red.  It 
is  readil)'  understood  why  the  house  phj^sician,  out  of  consideration  for 
the  other  children,  ordered  removal  to  the  hospital.  On  examination 
by  the  nurse,  there  was  no  trace  of  a  rash;  it  reappeared  on  every 
examination  by  a  phj'sician. 

Similar  difficulties  are  encountered  when  fever  is  present,  in  addi- 
tion to  hyperiemia  due  to  crying. 
II— 19 


290  THE   DISEASES   OF   CHILDREN 

Ludovica  S.,  eighteen  months  old.  Illness  began  with  fever,  vom- 
iting and  convulsions.  Mother  noticed  no  rash.  On  e.xamination,  the 
child  cried  violently  and  a  confluent  erythema  appeared  on  the  trunk 
and  extremities.  Though  the  cavity  of  the  mouth  was  pale,  the  child 
was  admitted  to  the  scarlet  fever  ward.  Temperature  40.2°  C.  (104.4° 
F.).  In  the  evening  the  temperature  was  normal;  no  rash.  Two  days 
later,  mild  typical  scarlatina.  In  this  case  the  mistake  might  have 
been  avoided  had  the  absence  of  a  pharyngitis  been  considered.  The 
fever  probably  was  due  to  some  digestive  disturbance. 

3.  Be  Careful  with  Young  Children. — In  children  under  one  year, 
one  must  be  very  conservative  about  the  diagnosis  of  scarlet  fever. 
Here  mistakes  occur  most  easily,  if  one  considers  onlj-  the  skin.  Of  the 
1059  scarlet  fever  cases  which  we  have  observed,  there  was  none  under 
four  months  of  age. 

4.  Erythema  from  Bandages. — ^The  irritation  of  the  skin  produced 
by  compresses,  is  confined  to  the  parts  where  they  were  applied;  hence 
the  extremities  are  usually  uninvolved.  [Flannel  underwear  frequently 
produces  a  punctate  rash  resembling  scarlet  fever  ;  it  is  limited  by 
the  extent  of  the  flannel. — LaF.]  Heat  rashes,  also,  afl'ect  the  trunk 
by  predilection. 

5.  Heat  Rashes. — ^The  infectious  exanthemata  most  frequently 
call  for  differential  diagnosis,  if  the  rash  does  not  closely  follow  the 
text  book  descriptions.  The  student,  who  sees  only  typical  cases  of 
scarlet  fever  and  measles,  believes  that  nothing  could  be  easier  than  to 
differentiate  them.  But  both  rashes  are  extremely  variable;  scarlet 
fever  especially  ma}'  assume  types  which  are  very  similar  to  the  papu- 
ular  forms  of  measles.  If,  in  addition,  coryza  and  conjunctivitis  are 
present,  as  occurs  in  the  more  severe  cases,  the  diagnosis  may  become 
very  difficult,  especially  by  artificial  light. 

6.  Points  for  measles  and  against  scarlet  fever  are: 

Measles. — 1.  Catarrhal  symptoms  and  a  prodromal  stage  of  sev- 
eral days'  duration;  sneezing,  coughing,  agglutination  of  the  eyelids 
before  the  appearance  of  the  rash. 

2.  The  finding  of  Koplik's  spots,  which  absolutely  prove  measles; 
also  a  large  macular  rash  of  the  palate. 

3.  Exanthema  in  the  regions  of  the  mouth  and  nose. 

4.  After  the  disappearance  of  the  rash,  pigmentation  in  patches. 
For  scarlet  fever  and  against  measles. 

Scarlet  Fever.— 1.  Acute  onset  of  the  disease  with  vomiting  and 
sore  throat. 

2.  Intense  redness  and  exudates  in  the  throat,  strawberry  tongue. 

3.  Circumoral  pallor. 

4.  After  disappearance  of  the  rash,  desquamation,  nephritis, 
lymphadenitis. 


SCARLET  FEVER  291 

Such  marked  pigmentation  as  usually  occurs  after  measles  rarely 
follows  scarlet  fever.  The  spots  after  measles  may  be  seen  for  two 
weeks,  while  in  scarlet  fever  areas  of  yellowish  discoloration  may  be 
made  out,  more  diffuse  than  in  measles,  though  less  pronounced  in 
color. 

The  desquamation  of  measles  is  very  delicate  in  branny  scales. 

The  possibility  of  removing  skin  in  rather  large  flakes  always  sug- 
gests scarlet  fever;   it  is  also  opposed  to  ichthyosis. 

In  neglected  children,  who  have  not  been  bathed  for  a  long  time, 
a  bath  may  produce  loosening  of  the  skin,  which  may  simulate  desqua- 
mation, though  it  is  spurious,  not  real. 

7.  Against  rubella  and  erythema  infectiosum  are  :  High  fever, 
over  39°  C.  (102.2°  F.);  dense  efflorescence  and  pronounced  pharyngitis 
(mild  pharyngitis  also  occurs  with  rubella).  Swelling  of  the  post-cervical 
lymph-nodes  speaks  for  rubella. 

Erythema  Infectiosum. — Erythema  infectiosum  resembles  scarlet 
fever  in  its  distribution  about  the  face,  but  the  large  macular  rash  on 
the  outer  side  of  the  extremities  can  hardly  be  confused  with  scarlet 
fever  (Plate  13).  In  case  of  a  typical  diffuse  rubella  rash,  the  diagnosis 
is  easy.  But  if  the  rash  is  small  and  macular,  the  case  may  be  diag- 
nosed as  scarlet  fever. 

Heimich  M.,  thirteen  years  old.  Day  before  yesterday,  malaise, 
fever,  headache.  To-day,  rash.  Bright  red,  small  macular  rash,  espe- 
cially on  the  flanks  and  legs.  Over  same  regions  numerous  reddened 
folUcles;  poorly  defined  exanthem  on  trunk  and  arms.  Pharynx  mod- 
erateh^  injected.  Early  diagnosis  of  scarlet  fever  proved  incorrect ;  on 
the  fono\^ing  morning  (fourth  day),  rash  had  disappeared.  Pharynx 
sHghtly  reddened,  no  strawberry  tongue.  Yeflow  spots  on  one  tonsil: 
no  desquamation;  no  sequela^;  sister  of  patient  passed  through  typical 
rubella. 

8.  Medicinal  rashes,  especially  following  the  use  of  atropine  and 
aspirin,  are  said  to  resemble  scarlet  fever.  The  changes  in  the  pharynx 
in  scarlet  fever  are  of  greatest  importance  in  diagnosis. 

9.  After  injection  of  serum  in  diphtheria,  scarlatiniform  rashes 
are  observed  in  rare  cases.  If  the  diphtheritic  pharyngitis  still  exists, 
these  rashes  can  hardly  be  distinguished  from  scarlatina,  so  that  the 
possibihty  of  differentiation  has  been  considered  doubtful.  The  follow- 
ing case  proves  that  these  rashes  are  not  always  scarlet  fever. 

K.  Emihe,  eight  years  old.  Diphtheria.  Injection  of  antitoxin.  On 
the  same  day  a  small  macular,  bright  red,  universal  erythema:  was 
transferred  to  scarlet  fever  ward,  when  sha  acquired  a  genuine  scarla- 
tina three  days  later. 

Before  the  appearance  of  the  rash,  scarlatinal  pharyngitis  often 
requires  a  diagnosis,  and  must  then  be  differentiated  from  other  pharyn- 


292  THE   DISEASES   OF   CHILDREN 

geal  inflaminations.  Tliis  is  especially  true,  because  scarlet  fever  need 
not  be  acconiiaanied  liy  a  rash.  Besides,  true  diphtheria  may  be  asso- 
ciated mth  scarlet  fever. 

Diphtheria. — Low  temperature,  insidious  onset  and  hoarseness 
favor  dijihtheria  and  are  opposed  to  scarlet  fever  and  follicular  ton- 
silhtis.  For  scarlet  fever  are:  Initial  vomiting  and  marked  injection 
and  swelling  of  the  pharynx. 

The  localization  of  exudates  upon  the  uvula  and  anterior  pillars  of 
the  fauces,  as  well  as  a  white  color  of  the  patches,  do  not  exclude  a  pure 
scarlet  fever  infection,  but  point  toward  diphtheria  (see  p.  275J.  The 
finding  of  abundant  streptococci  in  the  exudate  speaks  against  diphtheria. 

In  the  later  stages  of  the  disease,  when  the  rash  has  disappeared 
and  desquamation  not  yet  set  in,  the  remnants  of  the  pharyngeal  exu- 
date may  closely  resemble  old  diphtheritic  exudates.  In  such  cases  the 
presence  of  the  strawberry  tongue  is  important,  as  well  as  eroded  con- 
tours of  the  pillars  of  the  fauces,  which  are  peculiar  to  the  inflammatory 
necrosis  of  scarlatina.  The  diphtheritic  process  does  not  produce  losses 
of  substance. 

Aside  from  scarlet  fever,  this  tendency  to  necrosis  in  pharyngeal 
affections  exists  only  in  sypliilis  and  advanced  tuberculosis. 

Leopoldine  v.,  twelve  years  old.  Admitted  to  hospital  vnth  diagno- 
sis of  scarlet  fever.  Shows,  in  addition  to  cachectic  desquamation  on  the 
extremities,  soft  yellow  confluent  exudates  on  the  cracked  and  eroded 
tonsils,  surrounding  which  the  mucous  membrane  is  anemic.  The 
diagnosis  was  based  on  the  presence  of  large  chronic  indolent  lymph- 
node  enlargements  of  the  neck,  and  the  discovery  of  advanced  pul- 
monary phthisis.  The  diagnosis  was  confirmed  by  the  demonstration 
of  tubercle  bacilli.     Result  fatal. 

Prognosis. — The  prognosis  of  scarlet  fever  is  one  of  the  most  diffi- 
cult to  determine.  Mistakes  happen  even  to  the  most  experienced 
physician.  It  may  be  stated,  as  a  general  rule,  that  during  the  first 
days,  in  spite  of  the  consideration  of  all  factors,  one  is  Ukely  to  fall  into 
the  error  of  regarding  the  case  as  milder  than  it  eventually  proves  to 
be.  This  depends  upon  the  fact  that  the  tendency  of  the  disease  to 
progress,  especially  in  its  infectious  manifestations,  is  not  always  evi- 
dent in  the  early  phases.  On  the  third  day  one  notes  to  some  extent 
the  intensity  of  the  primary  disease.  Therefore,  the  rule  should  be 
established,  not  to  make  a  prognosis  on  the  first  or  second  days,  and  even 
later  the  prognosis  should  be  confined  to  the  disease  itself.  The  sequels 
of  scarlet  fever  cannot  be  foretold,  because  their  appearance  and  course 
and  seventy  are  absolutely  independent  of  the  symptoms  of  the  primary 
infection.  Therefore,  the  possibiUty  of  sequelae  must  be  borne  in  mind, 
even  in  the  mildest  cases.  It  is  wsest  never  to  set  tip  an  absolutely  favor- 
able prognosis  for  scarlet  fever.     It  is  true  that  one  has  in  one's  favor  the 


SCARLET  FEVER  ii)S 

rule  of  chances;  since  only  a  minority  of  cases  develop  sequela?  and  of  these 
only  a  small  percentage  terminate  fatally.  The  size  of  tliis  percentage 
depends  on  the  character  of  the  existing  epidemic. 

One  who  would  have  guaranteed  a  favorable  outcome  in  the  follow- 
ing case  would  have  been  stultified  by  its  course.  Karl  Z.,  twenty-one 
months  old.  Admitted  on  third  day,  with  ma.ximum  temperature  of 
38.5°  C.  (101.3°  F.).  Morning  remission  to  37.7°  C.  (99.9°  F.).  Mod- 
erate rash;  pharyngeal  injection  without  exudate;  defervescence  by 
lysis.     After  the  sixth  day  temperature  below  38°  C.  (100.4°  F.). 

Seventeenth  day,  bloody  urine. 

Nineteenth  day,  temperature  40  C.  (104°  F.);  increase  of  albumin 
in  urine  and  of  oedema. 

Twenty-sixth  day,  abdominal  swelhng;  injection  of  umbiUcus; 
constant   emesis. 

Twenty-seventh   day,   death  (nephritis,  peritonitis). 

According  to  the  procedure  of  Moser,  we  distinguish  favorable 
cases  (Prognosis  I  and  II);  doubtful  (III);  fatal,  (IV).  This  division 
results  from  an  attempt  to  determine  the  probable  course  of  the  disease 
on  the  day  of  admission  to  the  hospital.  By  comparison  of  the  actual 
course  with  the  probable  outlook,  we  learn  the  prognostic  value  of  the 
various  symptoms. 

Toxic  Form. — Chnically,  two  forms  of  severe  scarlet  fever — wliich, 
however,  often  merge  into  one  another — may  be  distinguished,  depend- 
ing on  the  prominence  of  the  infectious  and  toxic  symptoms.  The  toxic 
form  is  characterized  by  high  fever,  intense  rash,  often  with  the  addition 
of  maculopapular  efflorescences  (double  exanthema);  conjuncti\'itis, 
rapid  respiration  and  pulse.  Cold  extremities  and  cyanosis  may  appear 
as  evidences  of  heart  weakness.  The  nervous  system  is  markedly 
affected  in  the  shape  of  apathj'  and  restlessness,  which  may  progress, 
on  the  one  hand,  to  coma;  on  the  other,  to  delirium,  jactitations  and 
convulsions.  In  these  cases  we  also  encounter  continued  vomiting  and 
green,  fetid,  diarrhceal  stools. 

These  foudroyant  symptoms  may  develop  rapidly  and  lead  to  a 
fatal  termination  within  twenty-four  or  forty-eight  hours,  even  before 
the  appearance  of  a  distinct  rash. 

Rosa  T.,  nine  j^ears  old.  Five-year-old  brother  had  suffered  from 
scarlet  fever  in  mild  form  five  weeks  previously.  Rosa,  on  July  6th, 
had  sore  throat;  on  the  7th  p.  m.  vomiting;  night  of  7th  to  8th  was 
restless;  had  diarrhoea;  at  9  x.  m.  convulsions,  jactitations.  At  10  p.  m., 
admitted  to  hospital;  stupor,  running  pulse,  stertorous  respiration; 
general  cyanosis.  Death,  fifteen  minutes  later.  Autopsy  :  Petechial 
injection  of  a  soft  palate  antl  pharynx ;  tonsils  enlarged  ;  purulent 
exudate ;  splenic  tumor ;  swelling  of  mesenteric  lymph-nodes.  Pure 
culture  of  streptococci  from  heart's  blood. 


294  THE   DISEASES   OF   CHILDREN 

The  nervous  symptoms  may  become  so  prominent  that  the  disease 
gives  the  impression  of  a  severe  cerebral  affection. 

Joseph  W.,  four  years  old.  In  evening,  sudden  diarrhoea  and  emesis; 
fever.  Rash  the  next  morning.  Numerous  light  green,  fetid,  liquid 
stools,  containing  mucus.  Afternoon:  Stupor,  delirium.  Evening:  Sent 
to  hospital  as  meningitis.  Temperature  40.7°  C.  (105.3°  F.);  pulse  150. 
Pronounced  cyanosis  of  extremities,  stupor,  twitching  of  muscles  of  face. 
Strabismus,  chewing  movements,  unequal  innervation  of  the  facial 
nerves,  jactitations,  hypera-sthesia ;  conjunctivitis ;  small  macular, 
bright  red  rash,  irregularly  spread.  Slight  pharyngeal  involvement 
(lacunar  patches);  nose  clear;  200  c.c.  Moser  serum  (24  hours  after 
onset).  During  the  night  all  cerebral  irritative  symptoms  vanished; 
temperature  dropped  to  38°  C.  (100.4°  F.);  pulse  128.  Following  after- 
noon, 38.9°  C.  (102°  F.).  On  next  day,  afebrile,  pulse  100.  Convales- 
cence uncompUcated.  We  attribute  the  termination  of  the  serious 
symptoms  in  this  case  to  the  use  of  the  serum. 

Richard  L.,  three  years  old.  Afternoon,  sudden  vomiting,  four 
times.  High  fever.  At  night  loud  crying.  Rash  on  the  following 
morning.  At  noon,  small  macular,  bright  red  rash;  lacunar  tonsiUitis; 
moderate  coolness  of  extremities.  Temperature,  40°  C.  (104°  F.).  At 
night  very  restless;  nauseated.  On  following  day,  double  exanthema; 
conjunctivitis;  pulse  160;  temperature  40.7°  C.  (105.3°  F.).  Purulent 
rhinitis.    Two  hundred  c.c.  ordinary  horse  serum. 

Fourth  day:  Cyanosis  pronounced;  temperature  40.9°C.  (105.6°  F.). 
Patches  increased  in  size.  At  autopsy,  were  found  in  addition  to  the 
ulcerative  changes  in  the  pharynx,  similar  fibrinous  exudates  upon 
the  gastric  mucosa,  and  delicate  fibrinous  deposits  on  pleura  and  peri- 
cardium. 

This  case,  which  showed  a  severe  pharyngeal  inflammation  and  a 
necrosis  of  the  mucous  membrane  of  the  stomach — an  absolutely  unique 
finding — presents  a  transition  to  those  severe  forms  of  scarlatina  which 
are  distinguished  by  increasing  infectious  phenomena  in  the  later  days 
of  the  disease  (not  before  the  third).  Among  these  phenomena  are 
severe  pharyngeal  changes  with  secondary  involvement  of  the  nose  and 
ear;  also  the  progressive  adenitis  and  periadenitis.  Such  cases  termi- 
nate fatally  at  the  end  of  the  first  or  in  the  second  week,  with  pysemic 
symptoms.  Some  may  be  protracted  for  weeks  (see  Karl  St.,  p.  275). 
This  and  the  following  case  may  be  taken  as  types  of  the  infectious 
form: 

Leopoldine  E.,  five  years  old.  On  fifth  day  of  disease,  temperature 
40°  C.  (104°  F.)  ;  apathy ;  coolness  of  extremities  ;  pronounced  rash 
with  subicteric  tint ;  conjunctivitis  and  dacryocystitis.  Intense  in- 
volvement of  nose  and  pharynx.  Fetid  breath.  More  favorable  symp- 
toms were  good  pulse  and  clear  mind.     Serum  injection.     Pharyngitis 


SCARLET  FEVER  295 

and  associated  lymphatic  enlargement  advance;  otitis  media  develops; 
fever  constant  between  39°  C.  and  40°  C.  (102.2°  F.  and  104°  F.).  On 
fourteenth  day,  aspiration  pneumonia,  ending  fatally  on  the  nineteenth 
day,  with  evidences  of  meningeal  involvement  and  gangrene  of  the  lung. 

Real  py(zmic  distribution  with  multiple  abscesses  occurred  only  in 
very  few  cases  in  the  epidemic  which  we  observed. 

Franz  H.,  six  years  old.  Admitted  on  seventh  day  of  illness.  Be- 
sides distinct  desquamation  on  the  buttocks,  the  skin  is  still  intensely 
scarlet,  with  a  suggestion  of  brown.  Disappears  only  slightly  on  pressure. 
(This  long  continuation  of  the  rash  in  itself  indicates  an  unfavorable 
course).  Purulent  nasal  discharge,  ahe  excoriated.  At  the  opening 
of  the  right  naris,  several  membranes  may  be  removed  by  forceps  and 
contain  streptococci.  In  the  cavity  of  the  mouth,  tough  mucus;  marked 
fetor  ex  ore.  Tongue  thickly  coated,  pharynx  swollen  and  covered  by 
smeary  exudate.  \'oice  very  hoarse.  The  swelling  of  the  cervical 
glands  is  so  indurated  and  extensive  that  the  head  is  held  backwards 
as  in  cerebrospinal  meningitis.    Pulse  156;  soft,  irregular.    Prognosis  IV. 

On  ninth  day,  infiltration  on  inner  surface  of  right  arm.  On  tenth 
day,  swelling  and  redness  of  dorsum  of  hands;  deep  fluctuation  in  infil- 
trated parts.  Temperature  at  first  is  relatively  low  (39°-39.4°  C; 
102.2°  to  103°  F.);  on  tenth  day,  rather  sudden,  ante-mortem  rise  to 
42.2°  C.  (108°  F.).  At  autopsy  there  were  general  pytemic  findings: 
Ulcerative  tonsillitis,  pharyngitis  and  laryngitis,  acute  hyperplasia  and 
suppuration  of  cervical  lymph-nodes,  mediastinitis,  embolic  nephritis, 
metastatic  abscesses  of  right  arm  and  backs  of  both  hands.  Parenchy- 
matous degeneration  of  viscera;  ecchymoses  in  pleura.  Pure  culture  of 
streptococci  in  heart's   blood. 

The  determination  of  a  prognosis  depends  on  the  consideration  of 
the  favorable  and  unfavorable  symptoms.  We  give  Prognosis  I  in  cases, 
when  there  is  not  a  single  unfavorable  symptom.  These  cases  include 
about  50  per  cent,  of  all  of  the  Vienna  epidemic. 

Under  Prognosis  II  are  classed  cases  in  which  one  or  another 
unfavorable  symptom  is  quite  pronounced  (high  temperature  and  high 
pulse  rate,  with  otherwise  slight  symptoms,  or  pronounced  pharyn- 
gitis without  involvement  of  the  nose). 

A  doubtful  prognosis  (Prognosis  III)  is  given  in  cases  in  which 
there  are  signs  of  heart  weakness  (cold  extremities  or  cyanosis),  marked 
involvement  of  the  nervous  system  or  gastro-intestinal  tract  (contin- 
uous vomiting,  green  stools),  or  double  rash,  or  when  the  infectious 
phenomena  in  the  throat  are  very  marked. 

The  age  must  also  be  considered.  Other  things  being  equal,  in 
the  infectious  form,  children  under  three  years  of  age  are  more  prone 
to  succumb  than  older  ones.  We  give  several  instances  in  which  we 
concluded  to  give  Prognosis  III. 


296  THE    DISEASES   OF   CHILDREN 

Gabriele  W.,  one  and  a  half  years  old.  Unfavorable  symptoms 
after  eighteen  hours:  Temperature  40°  C.  (104°  F.);  intense  rash; 
lassitude;  age.  Favorable  symptoms:  Nose  uninvolved;  mild  pharyn- 
gitis. 

Hermann  K.,  seven  years  old.  Symptoms  after  thirty-six  hours: 
Considerable  nasal  and  pharyngeal  involvement;  coolness  of  e.xtremi- 
ties  ;  cyanosis  ;   double  rash  ;   lassitude. 

Hermine  G.,  seven  years  old;  unfavorable  symptoms  thirty-six 
hours  after  onset:  Rapid  development  of  symptoms;  cold  extremities; 
cyanosis.  Temperature  40.4°  C.  (104.7°  F.).  Pulse  IGO.  Mentally 
confused;   rigidity  of  neck.     Favorable  symptom:   Nose  free. 

We  rarely  give  an  absolutely  unfavorable  prognosis  (IV);  we  do  so 
only  when  a  large  number  of  severe  symptoms  occurs  in  a  combination, 
which  is  shown  by  experience  to  lead  to  a  fatal  result. 

Stefan  V.,  six  years  old.  Coma,  grinding  of  teeth,  double  rash, 
fiuid  .stools;  temperature  40.3°  C.  (104.6°  F.);  pulse  180;  dilatation  of 
heart  (three  days  after  onset,  toxic  form).     (For  course,  see  p.  287). 

In  the  infectious  form,  which  develops  gradually,  it  is  impossible 
to  foretell  a  fatal  termination  on  the  first  days;  the  same  holds  for  the 
toxic  form.     This  is  possible,  however,  on  the  later  days. 

Franz  H.,  six  years  old.  Severe  pharyngeal  and  nasal  infection; 
bilateral  otitis;  enormous  cervical  lymph-node  enlargement;  conjunc- 
tivitis; cold  extremities;  cyanosis;  mental  confusion;  pulse  160,  small. 
(For  course,  see  p.  288). 

Pneumonia  is  a  complication  which  considerably  aggravates  the 
outlook ;  we  mention  it  here  for  the  first  time,  because  it  does  not 
belong  to  the  picture  of  scarlet  fever  itself.  In  contradistinction  to 
measles,  in  which  the  mucous  membrane  of  the  respiratory  tract  is 
especially  involved  by  the  disease  itself,  and  by  its  complications,  the 
respiratory  tract  in  scarlet  fever  is  usually  spared.  But  when  pneumonia 
does  appear  in  the  early  days  of  the  disease,  the  case  must  be  considered 
as  very  serious,  even  if  all  the  other  symptoms  are  mild,  because  either 
the  pneumonia  itself  terminates  fatally  or  it  suppurates,  infecting  the 
pleura  and  resulting  in  a  mahgnant  empyema. 

It  is  impossible  to  decide  to  what  extent  these  pneumonias  are 
etiologically  associated  with  scarlet  fever.  When  they  suppurate, 
streptococci  are  almost  invariably  found  in  the  empyema  pus. 

Joseph  L.,  two  and  a  half  years  old.  Admitted  on  the  sixth  day 
with  slight  scarlatinal  symptoms  and  pneumonia  of  the  right  upper  lobe. 

Eleventh  day:   Pyopneumothorax. 

Twelfth  day:   Death. 

At  autopsy  :  Confluent  lobular  pneumonia  of  right  upper  lobe, 
with  focus  of  necrosis  as  large  as  a  walnut  near  the  apex.  Pyopneu- 
mothorax. 


PLATE   17. 


a      Gangrene  after  scarlet  fever  in  a   syphilitic  chikl. 

b.     Enanthem   on   the  intestinal  mucosa  with  follicular  hemorrhages  in  septic  scarlet  tever. 


SEQUELiE,  INFECTIOUS  CHARACTER  AND  TREATMENT 
OF  SCARLET  FEVER 

BY 

Dk.  B    SCHICK,  OF  Vienna 

TRANSLATED  BY 

Dr.  ISAAC  A.  ABT,  Chicago,  III. 


SEQUELJE  OF  SCARLET  FEVER 

The  danger  of  scarlet  fever  has  not  disappeared  after  the  resolu- 
tion of  the  primary  infection  and  the  imnieiliately  associated  compli- 
cations. After  them  the  sequelae  may  delay  convalescence  for  weeks; 
in  rarer  instances,  they  may  even  result  fatally. 

A  feature  common  to  all  of  the  sequelae  is  the  uniform  time  of  their 
appearance.  Postscarlatinal  diseases,  which  can  be  distinguished  from 
the  primary  infection,  appear  at  the  earliest  on  the  twelfth  day  ;  at  the 
latest,  during  the  sixth  week  after  the  onset.  Most  often  they  begin 
during  the  third  week. 

The  days  of  onset  of  the  cases  of  postscarlatinal  lymphadenitis, 
observed  by  us  during  the  last  four  years,  arranged  by  weeks  following 
the  inception  of  the  disease,  are  as  follows: 

2nd  week  (12th  to  14th  day) 3 

3rd  week 38 

4th  week 24 

.5th  week 5 

6th  week 1 

After  the  end  of  the  fourth  week,  the  frequency  of  sequehr  rapidly 
decreases.  Strictly  considered,  there  is  no  absolute  certainty  till  the  seventh. 
loeek.  The  latest  appearance  of  lymphadenitis,  which  we  have  seen, 
occurred  on  the  forty-first  day.  A  second  common  peculiarity  is  the 
acute  onset  of  the  symptoms,  with  a  remittent  type  of  fever,  which,  as 
in  the  primary  disease,  has  a  tendency  to  drop  by  lysis. 

Oscar  B.,  eight  years  old  (see  p.  29S,  Fig.  5-1).  Mild  scarlatinal 
rash,  with  follicular  tonsillitis;  maximum  temperature,  39.2°  C.  (102. (1° 
F.);  destjuamation.  No  temperature  from  fifth  to  seventeenth  day. 
On  seventeenth  day,  afternoon,  38°  C.  (100.4°  F.);  on  eighteenth, 
38.8°  C.  (101.8°  F.);  a  tender  lymph-node  on  angle  of  jaw,  as  large  as 
a  bean.  Urine  contains  no  albumin  (some  appeared  later).  Nineteenth 
day:  Maximum  temperature,  39.2°  C.  (102. ()°  F.).  Lymph-node  a  little 
larger;   in  afternoon,  pains  in  right  ankle,  which  is  distinctly  swollen 

297 


1298 


THE   DISEASES   OF   CHILDREN 


and  reddened  in  spots  on  its  outer  surface.  Twenty-first  day,  tem- 
perature and  node  enlargement  diminished.  Ankle  joint  unchanged. 
On  the  twenty-second  day,  pains  in  ankle  and  fever  disappeared; 
on  the  twenty-third  day,  the  swelling  also  subsided. 

In  the  preceding  case,  lymphadenitis  was  associated  with  joint 
involvement.  We  actually  see  the  different  sequelae  occurring  alone 
or  in  manifold  combinations.  The  diseases  which  are  observed  are 
chiefly  nephritis  and  also  processes  which  appear  about  the  same  time 
as  the  nephritis,  such  as  lymphadenitis,  rheumatism,  endocarditis, 
simple    fever   without  demonstrable  cause,  and  scarlet  fever  relapses. 

Nephritis. — Nephritis  usually  sets  in  with  emesis,  anorexia  and 
fever.  The  face  becomes  strikingly  pale;  sometimes  the  first  symptom 
is  a  characteristic  puffing  of  the  e3'elids. 

Fig.  54. 


DryoFifiphs*  1  9  ^\  i"^  7  H                       1 

^   itt       ^22L_+„            2£ 

LsIlii'  _i3^  Is[i 

SiS 

-----T       - 

1   jlj  [T  pi^;i  p  \\t\% 

1    ,        ,     fir  rl 

\\\  e  mat  sin.  r 

If 

i 

jljj^ 

?r  Y^fM?i^'^^'!^>^'^fA":p^  "■ 

■~^W  1    I 

Ml  4_ 

yi  tp  lA  \t  itl!]!i 

/'  ■  ■ .  'x. 

10  t<  f.;  rl 

J^^ik 

LiB^^^^L 

Temperature  curve  in  scarlet  fever. 

The  change  in  the  kidneys  themselves  at  first  is  evidenced  by 
traces  of  albumin  in  the  urine;  two  or  three  days  later  the  urine  becomes 
bloody  and  rich  in  sediment.  Again,  nephritis  may  begin  with  hiema- 
turia,  as  in  the  following  case,  in  which  other  severe  symptoms  (lympha- 
denitis, high  fever)  precede  the  onset  of  the  illness. 

Initial  Hematuria. — Hermine  M.,  four  years  old.  Moderately  severe 
scarlatina  with  fever  up  to  the  sixteenth  day  of  illness,  dependent  upon 
rheumatism  and  otitis.  On  the  twentieth  and  twenty-first  days  again 
pains  in  the  joints;  temperature  then  normal  to  thirtieth  day.  At  4 
p.  M.,  temperature  37.1°  C.  (99°  F.),  cheerful;  6  p.  m.,  child  complains 
of  pains  in  the  region  of  the  angle  of  the  right  lower  jaw.  Temperature 
rises  within  four  hours  to  40.9°  C.  (105.6°  F.);   pulse  to  200.    Following 


SCARLET  FEVER  299 

morning,  38.4°  C.  (101.1°  F.);  at  angle  of  right  lower  jaw,  a  tender 
lymph-node,  as  large  as  a  bean,  surrounded  by  an  area  of  diffuse  swelling. 

On  the  thirty-second  day,  .\.  m.,  37.8°  C.  (100°  F.);  urine  contains 
no  albumin;  at  noon,  40.4°  C.  (104.7°  F.).  At  2  p.  m.,  bloody  urine 
in  small  quantity.  Vomited  several  times.  Appearance  of  transient 
rashes  in  large  patches.  Abundant  sediment  in  urine.  Intermittent 
fever  continues  to  the  thirty-fifth  day.  On  thirty-seventh  day,  blood 
disappears.  On  forty-sixth  day,  no  albumin  (maximum  amount  of 
albumin  was  on  tliirty-tliird  day:   one-half  per  cent.);    recovery. 

In  cases  in  which  ha>maturia  occurs  in  the  beginning,  this  symp- 
tom is  so  striking  that  the  nephritis  can  hardly  be  overlooked.  Even  in 
little  children,  who  urinate  in  bed,  the  change  in  the  urine  may  be 
recognized  by  brownish  spots  on  the  bed-clothes. 

The  urine  at  the  time  is  turbid,  sometimes  bright  red,  sometimes 
brownish  red  and  deposits  a  heavy  sediment.  The  brownish  red  color 
does  not  permit  the  diagnosis  of  a  nephritis  of  long  duration,  for  the 
urine  may  show  this  color  very  early.  The  sediment  consists  of  casts  of 
all  varieties  and  of  flaky  reil  blood  corpuscles. 

Chemical  examination  shows  the  urine  to  contain  soluble  albumin 
in  amounts  of  j  to  10  per  mille.,  rarely  more.  The  chemical  tests 
(heat,  acetic  acid,  potassium  fcrrocyanide)  should  never  be  omitted, 
because  traces  of  albumin  usually  precede  the  hsematuria  and  because 
albuminuria  may  be  the  onh'  sign  of  a  mild  nephritis. 

Another  symptom  is  diminished  amount  of  urine.  Without  hsema- 
turia, it  is  conspicuous  at  the  onset  only,  if  very  marked.  If  traces  of 
all^umin  have  been  demonstrated,  the  urine  should  be  collected  for 
twenty-four  hours,  because  observation  of  the  quantity  per  diem  is  of 
importance  prognostically. 

The  daily  registration  of  the  body  weight  is  also  valuable,  but  is  rarely 
practicable  in  private  practice.  The  amount  of  oedema  is  expressed 
by  the  body  weight,  and  its  increase  and  decrease  is  likewise  of  prog- 
nostic significance,  ffidema  appears,  unlike  the  congestive  oedema  of 
patients  with  cardiac  disease,  not  only  in  the  dependent  parts,  but  by 
preference  in  the  face  (eyelids),  in  the  feet  and,  in  boys,  in  the  scrotum. 
With  an  increase  of  the  oedema,  general  anasarca  develops  and  collec- 
tions of  fluid  appear  in  the  serous  cavities — ascites  and  hydrothorax. 
The  onset  and  disappearance  of  oedema  is  frequently  remarkably  rapid, 
as  in  the  following  typical  case  of  well-developed  nephritis. 

Robert  M.,  eleven  years  old  (see  Fig.  55,  p.  301).  Admitted  on  third 
day  with  intense  rash  and  slight  pharyngeal  involvement:  within  two 
days  fever  disappears.  OrcUnary  desquamation;  patient  feels  well  till 
sixteenth  day.  Sixteenth  day,  in  a.  m.,  urine  showed  traces  of  albu- 
min;  noon,  sudden  increase  of  temperature  to  39.2°  C.  (102.6°  F.);  at 
1  p.  M.,  emesis,  then  repeated  nausea;   lassitude;    face  puffy. 


300  THE    DISEASES   OF   CHILDREN 

Seventeenth  day:  1  kg.  (2.2  pounds)  increase  in  weight  since  yes- 
terday. Total  quantity  of  urine  for  yesterday  only  J  of  normal  (about 
400  CO.);  contains  i  per  niille  albumin,  no  sediment.  Heart  action 
slow.  Eighteenth  day:  Urinary  sediment  contains  numerous  granular, 
and  few  hyaline  casts;  puLse  72  (formerly,  between  90  and  120).  Twen- 
tieth day:  Maximum  body  weight;  oedema  amounts  to  3  kilograms 
(6.6  pounds);  urine  reddish;  sediment  is  abundant  and  contains  red 
blood  corpuscles  and  blood  shadows  in  addition  to  epithehal  and  gran- 
ular casts.  4  per  cent,  albumin.  Pulse  in  a.  m.,  96.  Headache  and 
lassitude  all  day.  At  11:4.5  p.  m.,  twitchings,  first  in  the  face,  later 
involving  the  entire  body,  especially  on  the  right  side.  Coma.  Face 
pale,  hps  cyanotic,  foam  at  mouth.  Pulse  1.50.  Convulsions  contin- 
ued and  venesection  was  performed  at  midnight.  About  200  c.c.  of 
blood  were  removed  from  the  right  median  vein.  Infusion  of  200 
c.c.  physiological  salt  solution  under  skin  of  abdomen.  After  that, 
convulsions  appeared  only  occasionally  and  at  longer  intervals.  Mild 
twitcliings  till  4  a.  m. 

Twenty-first  day:  Lassitude.  ]\Iind  clear.  Xow  and  then  very  slight 
twitchings  and  nausea.  15  to  20  green  hquid  stools,  containing  shreds 
of  mucus.  During  the  entire  day  the  patient  drank  only  \  litre  of  alka- 
line water.    Urine — total  quantity,  250  c.c,  bloody,  sediment  unchanged. 

Twenty-tlaird  day  :  Weight  1.7  kg.  (3j  pounds)  less.  Diarrhoea 
continues.  Urine  not  increased  in  quantity ;  abundance  of  blood. 
Beginning  on  the  twenty-fourth  day,  diuresis  increases  (without 
any  therapeutic  measure  except  venesection)  up  to  2,000  c.c.  on  the 
thirty-second  day.  After  twenty-ninth  day,  no  sediment;  after  thirty- 
second,  no  albumin.  Recovery.  Great  actual  increase  in  weight. 
Discharged  from  hospital  on  forty-ninth  day. 

The  danger  of  nephritis  lies  cliiefly  in  the  occurrence  of  ura-mia, 
which  sometimes,  as  in  this  instance,  sets  in  suddenly  in  apparently 
mild  cases.  Usuallj',  though,  serious  ura?mic  symptoms  are  preceded 
for  several  days  by  mild  disturbances:  persistent  headache,  severe 
abdominal  pains,  nausea,  fixed  stare,  slow  high-tension  pulse. 

While  these  symptoms  suggest  the  possibiUty  of  the  beginning  of 
epileptiform  m'temia,  the  asthmatic  type  is  preceded  by  frequent  respira- 
tion.   In  this  form,  consciousness  may  be  retained  till  death. 

Julius  R.,  three  years  old.  Mild  scarlatina.  After  eleventh  day, 
pulse  arrhythmic;  retarded  to  64  in  spite  of  fever  and  lymphatic  en- 
largement. 

Fourteenth  day:   Traces  of  albumin. 

Sixteenth  day:  1  per  cent,  albumin  ;  l)lood ;  ohguria;  increa.se.in 
body  weight. 

Eighteenth  day:  In  evening  dyspno-a,  orthopnoea,  blood  pressure 
165  mm.  (Gartner). 


SCARLET  FEVER 


301 


Twentieth  day:  Evening  temperature  41°  C.  (105.8°  F.);  pulse 
192,  increasing  dyspnoea,  cyanosis  of  mucous  membranes  ^\ithout  mental 
disturbance.    Foamy  sputum. 

Twenty-first  day:  Temperature,  40.6°  C.  (105.1°  F.).  p.m.:  Death 
during  clonic  convulsions. 

Dyspnoea  is  not  always  a  sign  of  pulmonary  codema.  It  may  depend 
on  bilateral  hydrothorax,  especiall}'  in  eases  in  which  pleuritic  signs 
are  associated. 

Adolf  G.,  five  years  old.  Scarlet  fever  three  weeks  ago.  Nephritis 
blood,  albumin  2  per  cent,  typical  sediment.  Below,  posteriorly  over 
the  lungs,  flatness  from  the  level  of  the  7th  dor.'^al  vertebra  down.  On 
both  sides  pleuritic  rul)  above  the  line  of  dulness.  Heart  dulness 
greatlj'  increased  in  both  directions.  (Right  border  of  sternum,  to  left 
beyond  the  apex,  wliich  is  in  nipple  line,  to  5th  interspace  outside  of 
nipple  line).  No  peri- 
cardial friction.  Edge 
of  Hver  extends  4  cm. 
beyond  the  costal  arch. 
Pulse  small,  soft,  of  low 
tension.  Dyspnoea  and 
cyanosis.  Diagnosis: 
Nephritis  with  second- 
ary inflammatory  pro- 
cesses in  both  pleura^. 
Hydropericardium; 
heart  weakness. 

On  the  use  of  digi- 
talis, the  condition  ra- 
pidly improves;  a  week 
later,  the  findings  about 
the  heart  were   normal.     Recovery  from  nephritis  in  seven  weeks. 

In  tliis  case  there  were  also  hydropericardium  and  signs  of  heart 
weakness.  In  spite  of  that,  recovery  occurred.  Another  much  more 
dangerous  compHcation  of  nephritis  is  pneumonia,  wliich  also  produces 
dyspnceic  symptoms.  .All  these  compHcations  must  be  ruled  out,  before 
cyanosis  and  dyspnoea  can  be  attributed  to  uripmia  of  an  asthmatic  tvpe. 

Ludwig  F.,  three  years  old.  .Admitted  six  weeks  after  scarlet  fever, 
with  nephritis.  Albumin,  1  per  cent,  marked  generaUzed  oedema. 
Temperature,  40.2°  C.  (104.4°  F.)  ;  dyspnoea;  no  transudates,  but 
pneumonia  of  right  upper  lobe.  Collapse  and  death.  Autopsy  showed 
chronic  parenchymatous  nephritis  -mth  haMiiorrhages,  eccentric  hyper- 
trophy of  the  left  and  dilatation  of  the  right  ventricle;  confluent  lobar 
pneumonia  of  right  lung. 

The    unfavorable   result   in   a   comphcating   pneumonia   probably 


Fig.  55. 

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Temperature  curve  in  scarlet  fever. 


302  THE    DISEASES   OF   CIIII>DREN 

depends  on  the  weakness  of  the  heart,  produced  by  nephritis.  When 
such  mahgnant  complications  (peritonitis,  p.  293)  do  not  appear,  or  if 
death  does  not  result  from  urtemia,  the  prognosis  of  scarlatinal  nephritis 
is  generally  good. 

Even  severe  cases  may  recover  in  two  to  three  weeks,  only  traces  of 
albumin  remaining  for  many  weeks.  Chronic  nephritis  very  rarely 
results,  and  one  must  not  lo,se  faith  in  ultimate  recovery,  even  when 
the  albununuria  lasts  for  a  long  time. 

Frieda  W.,  five  years  old.  Mild  attack.  On  fifteenth  day:  Lympha- 
denitis; albuminuria.  Two  days  later,  bloody  urine.  Maximum  albu- 
min and  blood  on  thirty- first  day  (over  2  per  cent.).  Thereafter  very 
gradual  diminution,  ffidema  disappeared  on  forty-fourth  day.  Until 
the  seventieth  day,  though  patient  felt  well,  there  was  a  trace  of  albu- 
min. Allowed  to  get  up  on  the  seventy-seventh  day.  Discharged  from 
hospital  on  eighty-second  day. 

The  outcome  of  nephritis  cannot  be  foretold  while  the  symptoms 
are  on  the  increase.  Neither  the  height  of  the  fever  nor  the  quantity 
of  albumin  and  intensity  of  the  oedema  permit  a  positive  conclusion  as 
to  the  course  of  these  three  symptoms;  the  height  of  the  fever  is  of 
least  significance.  On  page  298  we  described  a  case  in  wliich  tempera- 
tures of  40.9°  C.  (105.6°  F.)  were  observed  and  still  the  nephritis 
terminated  favorably  in  two  weeks. 

Of  all  factors,  the  daily  quantity  of  urine  gives  the  best  clue  as 
to  the  course.  The  smaller  the  amount  of  urine  and  the  longer  the 
duration  of  the  oUguria,  the  more  serious  does  the  case  appear.  The 
improvement  of  the  nephritis  is  therefore  indicated  by  an  increase  in 
quantity  of  urine;  the  urine  becomes  paler,  and  at  the  same  time  the 
blood  and  albumin  diminish.  Sometimes  the  favorable  turn  occurs  by 
crisis,  as  in  the  case  of  Robert  M.  (p.  299),  whose  urine  increased  rapidly 
from  150  c.c.  to  2000  c.c.  per  diem,  and  in  whom  a  rapid  loss  in  body 
weight  resulted  simultaneously. 

Lymphadenitis. — A  sequel  wliich  offers  a  favorable  prognosis  is 
pure  postscarlatinal  lymphadenditis. 

Anna  H.,  seven  years  old.  Moderate  scarlatina.  Normal  tempera- 
ture on  twelfth  day.  Nodes  at  angle  of  jaw  almond-sized.  Twenty- 
first  day:  a.  m.,  no  fever;  afternoon,  39.2°  C.  (102.6°  F.).  Pains  at 
angles  of  both  jaws.  Twenty-second  day:  Nodes  painful  at  these 
points,  enlarged  to  size  of  hazelnut.  In  the  course  of  the  day  pain 
disappeared.  General  condition  good.  Twenty-third  day:  Tempera- 
ture normal.     Nodes  only  bean-sized  and  not  tender. 

A  painful  swelling  with  fever  begins  at  the  angle  of  the  jaw. 
Usually  a  single  node  is  affected;  this  feels  hard  and  succulent.  Tender- 
ness is  the  most  prominent  of  the  subjective  symptoms.  The  fever 
usually  rises  in   the  afternoon,   often   attaining  a  considerable   height 


SCARLET  FEVER 


303 


Fig.  56. 


(102.2°  F.  to  104°  F.,  or  more).  On  the  following  morning  the 
temperature  usuaUy  drops  to  less  than  38°  C.  (100.4°  F.).  Fur- 
ther developments  are  subject  to  individual  variations.  Usually  the 
excellent  general  condition  forms  a  marked  contrast  to  the  high 
fever.  Only  in  a  few  cases  is  the  disease  introduced  by  vomiting, 
which  may  be  repeated.  In  such  instances,  the  cliildren  usually  become 
pale,  the  face  is  slightly  puffed,  they  lose  their  appetite,  are  restless 
at  night.  The  onset  of  a  nephritis  is  suggested,  but  the  urine  remains 
free  from  albumin. 

On  the  average,  the  disease  terminates  in  four  to  six  days.  Usually 
the  tenderness  disappears  first;  this  indicates  that  the  maximum  of  the 
disease  is  passed.  Then  the  induration  and  size  of  the  swelling  dimin- 
ish, the  afternoon  tem- 
perature decreases  and 
the  disease  terminates 
with  defervescence  by 
lysis. 

Wilhelm  Sch.,  six 
years  old.  Moderate 
scarlet  fever,  with  pri- 
mary lymphadenitis. 
On  eleventh  day,  fever 
has  disappeared. 
Nodes  at  angle  of  jaw 
as  large  as  an  almond. 

Eighteenth  day: 
p.  M.,  temp.,  39.4°  C. 
(103°  F.)  Nineteenth 
day:  a.  m.,  temp.,  38.2° 
C.  (100.8°  F.).   Tender 

nodes      at      ano'le       of  Temperature  curve  in  scarlet  fever,  complicated  by  lymphadenitis. 

right  jaw  are  as  large  as  a  plum,  two  as  large  as  beans.  Twentieth 
day:  39°  C.  (102.2°  F.)  to  37°  C.  (98.6°  F.).  Nodes  unchanged. 
Twenty-first  day:  39.3°  C.  (102.8°  F.)  to  37°  C.  (98.0°  F.).  Nodes 
smaller;  not  tender.  Twenty-second  day:  38.3°  C.  (101°F.)  to  37.7°  C. 
(100°  F.).  Twenty-tliird  day:  No  fever;  lymph-node  enlargement  loss; 
duration  of  disease,  six  days. 

The  swelling  of  the  lymph-nodes  rarolj'  reaches  a  high  grade:  pro- 
nounced infiltration  never  occurs,  as  in  primary  scarlatinal  infections. 
The  most  unfavorable  result  is  suppuration,  which  happened  only 
twice  in  73  cases.  In  this  event,  as  in  the  primary  lymphadenitis,  it  is 
advisable  to  wait  for  incision  until  complete  softening. 

Eleanore  M.,  six  years  old.  Moderate  scarlet  fever.  On  twelfth 
day  a  lymph-node,  previously  as  large  as  a  hazel-nut,  increases  in  size  to 


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304 


THE   DISEASES   OF   CHILDREN 


that  of  a  hen's  egg;  temp,  to  40.6°  C.  (105.1°  F.);  morning  remission  to 
37°  C.(  98.6°  F.).  Diffuse  swelling  of  right  submaxillary  region.  Gidema 
extends  to  the  cheeks.  Nineteenth  day:  Fluctuation.  Decrease  in 
temperature.  Twenty-first  day:  Incision,  followed  by  escape  of  abun- 
dant, thick,  greenish  yellow  pus,  containing  streptococci.  Rapid  recovery. 

We  found  a  pure  form  of  postscarlatinal  lymphadenitis  in  over 
7  per  cent,  of  scarlet  fever  cases.  It  follows  scarlet  fever  in  about  the 
same  frequency  as  nephritis. 

By  preference  the  disease  accompanies  the  beginning  of  a  neph- 
ritis or  precedes  it.  This  possibility  alone  tlisturbs  the  otherwise  abso- 
lutely favorable  prognosis  of  lymphadenitis;  lymphadenitis  is  not  to  be 
considered  as  serious  as  nephritis  and  other  sequelae,  but  its  appearance 


Fig.  57. 


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Temperature  curve  m  scarlet  fever,  complicated  by  endocarditia. 

rather   acts  as   a  warning,  that  convalescence  or  rather  the  period  of 
sequela  will  not  pass  uneventually. 

Like  nephritis,  lymphadenitis  is  of  value  in  determining  the  exist- 
ence of  a  past  scarlet  fever.  In  the  case  of  every  child  that  shows  an 
acute  unilateral  lymphadenitis  without  pharyngeal  changes,  one  must 
consider  this  possibility.  Therefore,  the  skin  must  be  examined  for 
desquamation  and  the  urine  for  albumin. 

Leobold  W.,  five  and  a  half  years  old.  Is  brought  to  dispensary  on 
account  of  a  pronounced  swelling  of  the  left  submaxillary  lymph-nodes 
(6  X  9  cm.).  Redness  of  skin  over  swelling.  On  examination,  a  branny 
desquamation  is  found  on  the  body;  the  parents  recall,  on  being  ques- 
tioned, that  three  weeks  previously  the  child  had  had  fever  and  a  rash. 

Other  Sequelae.— In  addition  to  joint  affections,  of  which  we  gave 
an    instance    (p.   297),   other   uncommon   sequelce  are:   fever  without 


SCARLET  FEVER 


305 


demonstrable  cause  and  endocarditis.  Of  the  latter,  we  saw  three  cases; 
all  of  them  followed  mild  cases  of  scarlet  fever,  and  resulted  in  the  pro- 
tluction  of  cardiac  lesions.  Their  onsets  occurred  on  the  twentieth, 
thirty-second  and  thirty-fifth  days. 

Hermine  K.,  five  and  half  years  old  (Fig.  57  on  p.  ."504).  Mild 
scarlet  fever.  Maximum  temperature,  38.1°  C.  (100.6°  F.).  Deferves- 
cence on  fifth  day.  No  fever  till  twentieth  day.  On  this  and  the 
following  days,  temperature  rose  to  39.4°  C.  (103°  F.),  with  striking 
tachycardia.  Pulse  150  in  the  evening,  with  temperature  39.1°  C. 
(102.4°  F.).  On  twenty-fifth  day,  apex,  which  had  been  in  the  fourth 
interspace  inside  of  nipple  line,  is  found  outside  of  mammillary  line  in 
fifth  interspace.  Heart  action  undulating.  At  the  apex  a  loud,  long 
systolic  murmur.    While  the  heart   findings  remain  unchanged  during 

Fig.  58. 


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Temperature  curve  iu  scarlet  fever — Endocarditis. 

the  following  days  except  for  an  increase  of  the  second  pulmonic  tone, 
fever  decreases  by  lysis  to  the  twenty-seventh  day.  On  the  tliirtj'- 
eighth  day  of  the  disease  patient  is  discharged  from  the  hospital  with 
a  typical  compensated  mitral  regurgitation. 

In  the  second  case,  the  endocarditis  was  preceded  by  two  periods 
of  fever,  the  cause  of  which  could  not  be  demonstrated. 

Anna  L.,  six  years  old  (Fig.  58).  Very  mild  scarlatina.  After  fifth 
day,  temperature  normal.  From  twenty-seventh  to  twenty-ninth,  and 
from  tliirtieth  to  thirty-second  days,  temperature  of  over  38°  C.  (100.3° 
F.);    normal  heart  findings. 

On  thirty-fifth  day,  sudden  afternoon  rise  to  39.4°  C.  (103°  F.); 
palpitation.  7  p.m.:  Apex  in  fifth  interspace,  almost  in  anterior 
axillary  line.  Loud  systolic  murmur,  strongest  in  pulmonic  area.  Pulse 
120;  small.  Remittent  fever  to  thirty-ninth  da)'.  Murmur  decreases 
in  intensity;  apex  moves  in  to  h  cm.  outside  of  mammillary  fine. 

11—20 


306 


THE    DISEASES    OF   CHILDREN 


Fig.  59. 


On  discharge  from  hospital  on  forty-fourth  day,  the  intensity  of 
the  murmur  is  less,  but  the  enlargement  continues. 

Rises  in  iemjyerature  without  organic  signs  or  symptoms,  as  before 
the  endocarditis  just  described,  are  found  alone  during  the  critical 
period.  Two  children  of  the  same  family  are  interesting  in  this  respect; 
both  had  mild  attacks  of  scarlet  fever  and  showed  similar  rises  in  tem- 
perature on  about  the  same  day  (Fig.  59). 

Emilie  G.,  six  years  old.    No  fever  on  sixth  day. 
Franz  G.,  five  years  old.    No  fever  on  eighth  day. 
Emilie  G.,  nineteenth  day:    Afternoon  temperature,  39°  C.  (102.2° 
F.).    Twentieth  day:  37.2°  C.  (99°  F.)  to  38.5°  C.  (101.3°  F.).    Twenty- 
first  day:  37.1°  C.  (98.8°  F.)  to  38.3°  C.  (101.1°  F.);  thereafter  no  fever. 

Franz  G.  Eighteenth 
day:  Afternoon  tempera- 
ture, 39°  C.  (102.2°  F.). 
Nineteenth  day:  3G.9°  C. 
(98.4°  F.)  to  39.3°  C. 
(102.8°  F.).  Twentieth 
day:  37.1°  C.  (98.8°  F.) 
to  38°  C.  (100.4°  F.). 

Finally,  diverse  and 
peculiar  rashes  must  be 
noted.  The  rashes  may 
be  large  macular  and 
transient  (Hermine  M., 
p.  298).  Once  we  saw 
an  eruption  of  maculopapular,  brownish  red  efflorescences  on  the 
extremities;  the  spots  at  tlie  beginning  were  about  1  mm.  in  diameter, 
and  incrcasetl  in  size  to  3  or  4  mm.  Finally,  they  had  a  small  central 
pustule.    After  lasting  several  days,  gradual  desiccation. 

Lembert  M.,  five  years  old.    Mild  attack.    No  fever  on  seventh  day. 
Seventeenth  day:  Temperature  39.5°  C.  (103.1°  F.). 
Eighteenth  day:   Albuminuria. 

Moderate  fever  on  the  following  days,  with  progressive  increase  of 
nephritis. 

Twenty-third  day:  Fever  of  pyremic  type  began  with  markedly 
intermittent  temperature  36.9°  C.  (98.4°  F.)  and  41°  C.  (105.8°  F.). 
This  persisted  for  four  days,  with  constitutional  disturbances:  collapse, 
mental  confusion,  chills. 

On  twenty-fourth  day,  eruption  of  the  above-mentioned  rash. 
Fresh  crops  till  the  twenty-eighth  day.  On  the  twenty-sixth  day,  there 
is  also  an  inflammatory  effusion  into  the  right  wrist,  left  ankle  and  right 
knee.  The  joint  swellings  continue  unusually  long;  nephritis  clears  up 
on  the  forty-ninth  day. 


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Temperature  curves  in  scarlet  fever. 


SCARLET  FKVER 


307 


The  pyemic  character  of  the  disease  at  the  time  of  eruption  leads 
to  the  presumption  that  the  changes  in  the  skin  were  embohc  in  character. 

Relapses.— The  diagnosis  of  a  rehipse  requires,  first  of  all,  the  posi- 
tive demonstration  of  a  previous  attack.  AVe  only  assume  that  there  is 
a  relapse  when,  during  the  first  attack,  there  was  present  a  scarlatini- 
form  rash  and  other  signs  (angina,  strawberry  tongue),  or  when  the 
scarlatinal  character  has  been  confirmed  by  desquamation,  nephritis  or 
the  infection  of  other  individuals. 

Pseudo-relapses. — Cases  which  are  admitted  to  .scarlet  fever  wards 
on  the  basis  of  a  mistaken  diagnosis  and  then  succumb  to  a  true  scarlet 
fever  are  naturally  pseudo-relapses. 


Fig.  60. 


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Temperature  cun'e  in  scarlet  fever,  showing  relapse  in  fifth  week. 

Karohne  R.,  ten  years  old.  Jlild  attack.  Typical  rash;  pharynx 
red;  strawberry  tongue;  desquamation.  No  fever  on  fourth  day.  On 
eighteenth  day,  left  tonsil  and  left  anterior  pillar  of  fauces  injected.  On 
the  former  a  wliite  patch  as  large  as  a  spht  pea.  Vomited  four  times  in 
the  course  of  the  day.  A.  m.,  pulse  90;  p.  m.,  140.  Sore  throat.  On 
the  following  morning  typical  rash.  Patch  larger;  injection  of  pharynx 
more  inten.se.  Tenderness  of  glands  at  angle  of  jaw.  Temperature, 
38.5°  C.  (101.3°  F.).  Increase  of  rash  to  fourth  day,  then  rapid  fading. 
Desquamation  repeated.     Recovery. 

The  onsets  of  the  relapses  observed  occurred  on  the  eighteenth, 
twenty-first,  twenty-second,  twenty-tliird,  twenty-sixth,  twenty-ninth, 
thirty-second,  thirty-seventh,  and  thirtv-ninth  days.  We  have  not  seen 
later  relapses. 

The  second  attack  may  be  considerably  more  severe  than  the  first; 
it  may  lead  to  all  kinds  of  complications,  and  be  followed  by  nephritis. 


308  THE    DISEASES   OF   CHILDREN 

Aurelia  Sch.,  eleven  and  a  half  years  old.  Positive  scarlet  fever. 
Desquamation.  On  thirty-seventh  day,  fresh  scarlatinal  rash,  with 
jtharyngitis  and  joint  pains.  The  second  attack  was  followed  by 
lymphadenitis,  otitis,  and  finally  by  a  severe  ha>niorrhagic  nephritis, 
sixty  days  after  the  onset  of  the  first  attack,  twenty-one  days  after 
the  onset  of  the  relapse. 

IMMUNITY,    INFECTIOUS    CHARACTER,    PROPHYLAXIS 

After  a  single  attack  of  scarlet  fever,  an  individual  usually  is  im- 
mune to  the  disease  for  the  rest  of  his  life.  But  now  and  then  undoubted 
second  attacks  do  occur. 

Therese  11.  First  attack  at  three  years  of  age.  Treated  in  hos- 
pital. Rash,  pharyngitis,  undoubted  desquamation.  Discharged  Jan. 
22,  1898.  Admitted  again  with  scarlet  fever  on  April  2,  1902.  Highest 
temp.,  38.8°  C.  (101.8°  F.)  on  second  day;  thereafter  defervescence  by 
lysis  till  tenth  day.    Desquamation. 

The  rarity  of  second  attacks  forms  a  marked  contrast  mth  diph- 
theria, in  which  fresh  infections  are  relatively  frequent  and  may  occur 
even  within  a  few  months  of  the  first  attack. 

It  is  not  possible  to  prove  whether  there  is  an  absolute  coiKjenital 
resistance  to  scarlet  fever.  At  any  rate,  only  a  small  part  of  the  children 
exposed  to  the  disease  are  infected;  adults  only  in  exceptional  cases. 
From  tliis  fact  it  seems  that  the  resistance  to  scarlet  fever  increases 
with  the  age.  Nor  is  it  justifiable  to  speak  of  a  regular  family 
predisposition,  for  if  it  does  occur  that  all  of  the  members  of  one 
family  run  a  similar  course,  there  are  many  more  famihes  in  which  a 
single  child  is  infected,  or  where  several  children  present  symptoms 
of  different  intensity. 

We  cannot  decide  whether  the  cause  for  a  similar  course  in  mem- 
bers of  the  same  family  should  be  conceived  as  a  family  trait  or  as  due 
to  the  nature  of  the  epidemic  (genius  epidemicus). 

Three  children,  Sch.,  acquired  a  mild  scarlet  fever.  All  developed 
lymphadenitis  about  the  eighteenth  day  (18,  18,  19),  with  consecu- 
tive nephritis.  Even  the  course  of  the  nephritis  was  exactly  the 
same.  Albumin  disappeared  from  urines  on  thirty-second  and  thirty- 
fourth  days. 

The  age  between  the  third  and  eighth  year  shows  the  greatest  sus- 
ceptibihty;  as  we  have  before  mentioned,  the  first  half  year  is  almost 
immune,  just  as  in  mea.sles,  while  whooping-cough  and  chphtheria  also 
attack  children  in  the  first  months.  The  resistance  shown  by  adults  is 
not  absolute;  they  also  may  be  affected,  especially  if  their  predisposi- 
tion is  increased  by  pharyngeal  troubles. 

Dr.  V.  P.  was  active  in  the  scarlet  fever  ward  from  Dec.  1,  1901,  to 
Feb.  1,  1902,  without  acquiring  the  disease.     At  the  end  of  March  he 


SCARLET  FEVER  309 

had  a  mild  attack  of  diphtheria,  and  returned  to  his  ser\ace  on  March 
27,  1902,  with  a  pharynx  wliich  was  still  injected.  Apr.  5,  1902: 
Yellowsh  gra}-  patches  on  congested  posterior  pharyngeal  wall  and 
posterior  pillar  of  fauces. 

April  6,  1902:   Scarlet  rash.    Serum  injection.     Recovery. 

Johanna  R.  did  not  acquire  the  disease  until  she  had  acted  as  nurse 
in  scarlet  fever  ward  for  two  years. 

A  predisposing  cause  could  not  l)e  elicited. 

The  dependence  of  the  actual  disease  upon  transient  prechsposing 
causes  differs  materially  from  mea.sles;  practically  everyone  who  has 
not  had  measles  is  infected  at  the  first  exposure,  no  matter  what  his 
age.  Upon  this  fact  depends  the  difference  in  epidemics  of  the  two 
diseases,  when  they  are  introduced  into  an  isolated  institution.  In  the 
case  of  measles,  the  disease  appears  in  great  numbers  on  definite  days 
and  the  epidemic  terminates  rapidly.  In  the  case  of  scarlet  fever,  the 
numljer  of  those  affected  increases  gradually  and  sporadic  cases  appear 
for  a  long  time. 

This  brings  us  to  the  mode  of  injection.  In  measles,  a  direct  close 
association  of  the  infected  individual  ^^•ith  a  patient  in  the  prodro- 
mal or  eruptive  stage  may  be  proven  in  each  instance.  Transmission 
by  a  third  person  or  by  objects  is  rare.  This  tends  to  show  that  the 
infective  agents  of  measles  are  rapidly  destroyed  outside  of  the  body 
of  the  patient. 

The  opposite  is  the  case  in  scarlet  fever.  Transmission  of  the  virus 
by  flakes,  clothes  of  scarlet  fever  patients  and  third  persons  certainly 
does  occur.  Whether  the  flakes  of  skin  contain  germs,  or  whether  the 
flakes  are  rendered  infectious  secondarily  by  secretions  (ear,  mouth), 
we  cannot  decide.  The  scarlet  fever  agent  may  remain  virulent  for 
years,  as  is  shown  by  numerous  cases  in  the  hterature. 

The  conditions  in  Vienna  are  poorly  suited  to  stuthes  of  this  kind, 
because  scarlet  fever  is  always  present  and  infection  of  unknown  origin 
cannot  be  absolutely  excluded.  This  also  holds  for  the  following  case, 
wliich  would  be  above  criticism,  if  in  a  place  free  from  the  disease. 

Rosa  B.,  aged  four  and  a  half  years,  acquired  scarlet  fever,  was 
taken  to  the  hospital,  and  died  there.  The  clothes  were  disinfected, 
but  the  mother  did  not  bring  the  fur  jacket,  shoes  and  cap  for  dis- 
infection, fearing  that  they  might  be  spoiled  by  the  steam.  She  kept 
these  tilings  in  a  box  in  the  attic  and  took  them  out  two  years  later, 
when  the  next  child,  Anna,  had  attained  the  age  of  the  deceased.  Four 
days  after  putting  on  these  clothes,  Anna  was  attacked  by  a  moderately 
severe  scarlatina. 

Probably  only  a  small  percentage  of  infections  are  actually  brought 
about  in  this  way.  By  far  the  greatest  number  of  infections  result  in 
scarlet  fever  also  from  direct  contact  with  a  patient;    of  course,  scarlet 


310  THE   DISEASES   OF   CHILDREN 

fever  without  a  rash,  angina  scarlatinosa,  also  plays  a  part  in  the  spread 
of  the  disease.  It  cannot  be  determined  which  stage  is  most  infective. 
In  hospital  infections,  it  is  usually  possible  to  save  the  nearest  bed 
neighbors  from  infection,  if  isolation  is  carried  out  on  the  first  day 
of  the  rash.  Transmission  during  the  period  of  incubation  (Vogi)  is 
therefore  improbable. 

A  great  trouble  with  scarlet  fever  is  that  it  is  impossible  to  say 
how  long  a  patient  is  injections.  We  have  seen  a  number  of  instances  in 
which  children  infected  members  of  their  family  after  a  stay  of  six  or 
eight  weeks  in  the  hospital,  in  spite  of  repeated  baths  and  disinfection 
of  clothes. 

Marie  S.,  twelve  years  old.  Mild  scarlatina.  On  dischare  from  hos- 
pital after  forty  days  no  more  desquamation.  Returned  to  family  on 
Dec.  11th.  On  the  14th,  Gisela,  thirteen  years  old;  on  the  18th, 
Margarete,  four  years  old,  and  on  the  23rd,  Albert,  six  years  old;  all 
were  attacked  by  a  mild  scarlatina. 

Whether  the  cause  lies  in  the  secretions  (nose,  pharynx,  ears),  which 
still  contain  virulent  germs,  or  dust  from  the  scarlet  fever  ward,  stick- 
ing to  the  body  and  clothes,  is  unimportant.  The  practical  conclusions, 
drawn  from  these  observations,  are  as  follows: 

Immediate  Isolation  of  the  Patient  and,  if  Possible, 
Conveya7ice  to  a  Hospital 

If  the  patient  has  been  in  contact  with  other  children  only  a  short 
time,  it  is  probable  that  they  have  not  yet  been  infected;  still  they 
should  be  considered  suspicious  for  eight  days. 

In  measles,  isolation  at  the  time  of  the  rash  is  superfluous,  because 
infection  regularly  takes  place  several  days  before  the  appearance  of 
the  rash,  and  includes  all  who  have  not  already  had  the  disease  and 
have  been  in  the  same  room  as  the  patient.  The  further  reason  for 
dispensing  with  isolation  in  measles,  viz.,  that  children  acquire  the 
disease  sooner  or  later,  does  not  hold  for  scarlatina.  The  probabihty  of 
its  acquisition  diminishes  from  year  to  year  with  the  increase  in  natural 
immunity. 

The  cliild  should  be  taken  to  a  hospital,  so  that  the  germs  may  not 
be  scattered  in  the  home,  since  they  may  remain  in  there  for  years,  as 
many  cases  in  the  hterature  prove.  The  longer  the  child  remains  at 
home,  the  more  infective  does  the  place  become. 

Even  when  the  child  is  brought  to  a  hospital  early,  the  home  should 
be  disinfected:  The  furniture  and  floors  should  be  washed  with  corrosive 
sublimate,  1:1000,  or  5  per  cent.  carboHc  acid;  the  playtliings  should 
be  destroyed.  The  washable  clotliing  should  be  washed  and  the 
dresses  disinfected,  preferably  by  steam;   leather  articles  by  formalin. 

The  physician  should  arrange  his  calls  so  that  he  sees  the  scarlet 


SCARLET  P'EVER  311 

fever  patients  after  all  the  others.  He  should  wear  a  hnen  gown  in  the 
room  and  thoroughly  wash  his  hands  on  lea%'ing  the  patient. 

In  spite  of  all  precautions,  the  physician  cannot  absolutely  guar- 
antee that  after  the  return  of  the  patient  from  the  hospital  or  from  iso- 
lation to  the  rest  of  the  family,  no  further  infection  will  occur  among 
the  children.  It  rarel}'  happens,  however,  if  the  above  precautions  are 
observed. 

The  infective  agent  of  scarlet  fever  has  not  been  positively  demon- 
strated as  yet.  It  is  generallj'  known  that  in  the  pharyngitis,  in  all 
suppurative  processes  (lymph-nodes,  ear,  etc.);  and  in  all  organs,  as 
well  as  in  the  blood,  of  scarlet  fever  patients,  streptococci  can  usually 
be  found  in  pure  culture.  Some  believe  that  the  .streptococci  are  only 
secondary  to  an  unknown  primary  organism,  while  others  believe  the 
streptococci  themselves  to  be  the  actual  etiological  agents.  The  latter 
assume  that  the  scarlet  fever  streptococcus  is  a  .specific  variety,  and 
is  only  morphologically  similar  to  the  other  species  of  streptococci 
(puerperal  fever,  erysipelas,  etc.).  They  attempt  to  differentiate  it  by 
agglutination.  The  chain  of  proof  is  not  complete,  for  the  reason  that 
scarlatinal  infection  by  cultures  of  streptococci  has  not  been  knoicn  to 
occur  in  man,  nor  has  a  similar  disease  been  produced  beyond  question  in 
animals  by  such  cultures. 

There  must  be  great  differences  in  the  virulence  of  the  infective 
agent;  in  no  disease  does  the  "genius  epidemicus"  (type  of  the  epi- 
demic) play  such  a  role  as  in  scarlet  fever.  There  are  epidemics  in  which 
the  morbidity  and  the  mortality  are  very  slight,  while  in  others  30  to  50 
per  cent,  of  all  patients  succumb  to  the  disease. 

Instances  of  the  foregoing  are  the  following:  Reys,  of  Aachen, 
had  only  two  deaths  in  two  thousand  cases  in  the  past  years,  while 
during  the  same  time  the  mortaUty  in  Ser\'ia  and  South  Russia  amounted 
to  about  30  per  cent. 

The  scarlet  fever  ward  of  the  St.  Anna  Ivinderspital,  in  Vienna, 
during  the  years  1901-1904,  admitted  324,  337,  204  and  252  children 
respectively.  In  the  year  1905,  the  admission  to  Dec.  1st  amounted 
to  350.  Our  own  observations  cover  1059  cases,  admitted  since  April 
1,  1902.    All  case  histories  are  taken  from  this  material. 

TREATMENT 

General. — We  confine  ourselves  to  a  description  of  the  therapeutic 
measures  which  we  actually  practice,  and  on  whose  value  we  can  pass 
our  own  opinion.  We  shall  not  consider  that  part  of  the  treatment 
which  administers  drugs  merely  for  the  sake  of  doing  something,  since 
every  practicing  physician  knows  enough  harmless  di-ugs  to  use  for  that 
purpose. 


312  THE   DISEASES   OF   CHILDREN 


GENERAL    TREATMENT 

Every  case  of  scarlet  fever,  even  the  mildest,  is  kept  in  bed  for  four 
weeks,  and  is  given  no  meat  during  this  period.  Tliis  is  a  prophylactic 
measure,  whose  necessity  is  not  proven.  But  we  agree  with  Baginsky 
in  tlie  bchef  that  the  forms  of  nephritis  arising  in  cliildren,  so  treated 
in  the  hospital,  run  a  milder  course  than  the  nephritides,  in  wliich  the 
preceding  scarlet  fever  has  run  its  course  at  home  without  observing 
the  above  rules. 

The  prescription  of  a  meat-free  diet  as  a  prophylactic  measure  is 
based  entirely  on  theoretical  grounds.  We  doubt  whether  it  is  correct 
to  conclude  that,  because  of  the  unfavorable  action  of  extractives  on 
chronic  nephritis,  a  siinilar  danger  arises  from  the  use  of  meat  and  meat 
soups  in  acute  nephritis  or  even  in  a  preceding  scarlatina.  Empirically, 
it  is  true  that  children  take  the  meat-free  diet  well,  tolerate  it  very  well, 
and  that  the  milder  cases  even  show  a  decided  increase  in  weight. 

Diet. — We  do  not  give  a  pure  milk  diet,  but  from  the  beginning, 
depending  upon  the  appetite  of  the  clrild,  we  give  in  addition  to  boiled 
milk,  malt,  coffee,  and  cocoa  in  milk,  cereals  (farina,  rice  and  malted 
infant  foods),  desserts  (made  of  farina,  toasted  bread  and  steamed  rice), 
rolls  (white  bread)  and  butter.  Also  soups  (pea,  bean  and  potato  soups), 
and  stewed  fruit  (pears,  apples). 

When  milk  disagrees  and  causes  loss  of  weight,  we  do  not  hesitate 
to  give  meat,  even  in  the  presence  of  a  nepliritis  (boiled  beef,  ham), 
and  we  have  seen  no  deleterious  effects. 

We  do  not  give  alcohol  in  any  form  as  a  tonic,  but  use  it  only  as  a 
stimulant  in  severe  cases. 

It  is  self-evident  that  the  temperature  of  the  room  and  ventilation 
must  receive  attention.  There  is  no  reason  for  an  immoderate  fear  of 
catching  cold;  it  should  not,  at  any  rate,  prevent  us  from  washing  the 
chikh'en  and  changing  their  clothing. 

Baths. — We  do  not  employ  baths  therapeutically,  but  give  one  on 
admission  for  cleansing  purposes,  and  four  to  six  at  the  end  of  conva- 
lescence for  disinfection.    In  the  interim,  the  cliildren  are  simply  washed. 

Antipyresis.^With  temperature  over  39.5°  C.  (103°  F.)  we  use  no 
antipyretic  drugs,  but  cool  packs. 

Cool  Packs. — Half  of  a  sheet  is  dipped  in  water  of  a  temperature  of 
15°  to  20°  C.  (60°  to  70°  F.),  wrung  out  and  rolled  up,  beginning  with 
the  dry  portion.  The  moist  half  is  first  placed  against  the  body,  which 
it  should  cover  from  the  axilla;  to  the  crest  of  the  ilium.  The  compress 
is  changed,  depending  on  the  temperature,  every  hour  or  two,  or  may 
be  left  on  the  body  till  it  dries  out. 

Another  method  of  reducing  temperature  is  as  follows:  The  child 
is  placed  upon  a  dry  sheet,  a  cold  wet  sheet  is  folded  up  and  placed  upon 


SCARLET  FEVER  313 

the  chest  and  flanks.  Then  the  ends  of  the  dry  sheet  are  folded  over  it. 
This  method  is  more  convenient  for  frequent  changing  of  the  com- 
presses. 

[AVarm  baths  (90°-95°  F.)  reduce  temperature,  quiet  restlessness 
and  are  extensively  used  in  practice. — I.  A.  A.] 

In  severe  cases  one  should  always  observe  the  temperature  and 
color  of  the  hands  and  feet,  wliich  must  be  kept  outside  of  the  packs. 
If  the  extremities  become  cold  or  cyanotic,  the  compress  umst  be  re- 
moved at  once,  and  if  they  do  not  then  become  warm,  it  must  be  replaced 
by  a  dry  warm  sheet.  Hot  water  bottles  may  be  needed  for  warming 
the  extremities. 

On  account  of  the  fluctuations  of  temperature  in  the  severe  cases, 
we  avoid  cool  baths,  for  while  giving  such  baths  it  is  difficult  to  note 
the  temperature  of  the  extremities,  and  if  the  temperature  is  too  greatly 
reduced,  collapse  may  result.  The  treatment  with  baths  and  sprinkling 
with  cool  water  (70°  F.)  practiced  in  former  years,  has  been  abandoned 
because  it  proved  of  no  demonstrable  value. 

Rash  and  desquamation  require  no  treatment.  Inunction  with 
vaseline  or  lanolin-vaseline  hastens  the  process  of  desquamation,  espe- 
cially if  followed  by  warm  soap  baths.  In  the  hospital  we  employ 
inunctions  of  oily  substances  only  in  cases  when,  during  the  period  of 
desquamation,  the  skin  is  dry,  itches  and  shows  a  tendency  to  eczema. 

SYMPTOMATIC    TREATMENT 

In  the  treatment  of  pharyngeal  affections  we  must  distinguish 
the  mild  from  the  severe  cases.  Simple  injection  and  swelling,  or  follic- 
ular exudates,  require  no  treatment.  We  wrap  up  the  neck  in  cokl 
wet  cloths,  which  are  changed  every  three  hours  during  the  day,  and 
are  left  in  place  during  the  night.  We  allow  older  children  to  gargle 
with  any  of  the  common  antiseptic  mouth  washes  (1  per  cent,  potas- 
sium chlorate,  1  per  cent,  hydrogen  peroxide). 

In  the  severe  pharnygeal  infections,  especially  of  young  children, 
we  must  guard  chiefly  against  drying  of  the  mouth.  The  cavity  of  the 
mouth  must  be  frequently  cleared  of  mucus,  which  collects  continually, 
if  the  cliild  breathes  through  the  mouth  on  account  of  olistruction  of 
the  nasal  passages.  For  this  purpose  drinks  are  given  at  short  intervals. 
Lemonade,  cold  tea  and  tea  with  lemon  juice  are  taken  most  cheer- 
fully. During  high  fever,  we  use  wine  soup,  which  also  acts  as  an  alco- 
holic stimulant.  It  is  prepared  of  white  wine  and  water  in  equal  parts; 
to  the  mixture  the  yolk  of  one  egg  and  sugar  are  added. 

The  mouth  is  cleaned  three  or  four  times  a  day  by  an  injection  of 
cold  water  from  an  ear  syringe.  At  first  the  children  protest,  liut  later 
they  find  this  cleansing  very  pleasant.     During  the  process  the  child 


314  THE    DISEASES   OF   CHILDREN 

sits  on  tlie  la])  of  a  nurse,  hands  and  feet  are  held,  and  the  head  bent 
slightly  forward. 

We  do  not  use  any  other  active  measures;  we  avoid  especially 
insufflation  of  powders  into  the  pharynx,  and  painting  of  drugs  on  the 
inflamed  surface;  we  avoid  also  injection  of  carbolic  acid  and  irriga- 
tion of  the  nose. 

Ice  packs  are  unnecessary  in  mild  cases;  in  more  severe  ones  the 
children  often  become  cold.  This  has  forced  us  to  abandon  their  use. 
We  also  have  given  up  the  use  of  cracked  ice  internally.  When  the  lips 
are  parched  and  cracked  and  bleed  easily,  these  are  greased  with  3  per 
cent,  boric  acid  in  lanolin-vaseline.  The  same  mixture  is  employed 
for  fissures  at  the  angles  of  the  mouth,  which  in  severe  cases  may  show 
a  lardaceous  exudate.  In  rhinitis,  we  use  the  same  preparation,  when- 
ever the  nose  is  cleared,  thus  preventing  the  proiluction  of  eczema 
about  the  nasal  orifices  by  the  irritating  secretion.  We  also  introduce 
it  into  the  nasal  orifices  on  an  applicator,  if  the  children  are  not  excited 
by  the  treatment. 

In  all  these  therapeutic  measures  we  avoid  over  medication.  If 
the  child  is  asleep,  we  let  him  rest,  since  sleep  is  at  least  as  important  as 
any  part  of  our  treatment. 

Karl  Sch.,  eleven  years  old.  Was  treated  at  home  for  a  severe 
scarlatina.  The  illness  of  the  child  was  greatly  aggravated  by  a  mis- 
understanding of  the  physician's  orders.  The  child  was  annoyed  by 
treatment  day  and  night.  First,  the  neck  cloth  was  changed:  then  the 
ear  injected;  then  the  mouth  cleansed;  then  the  pack  renewed;  then  the 
child  was  forced  to  drink  against  his  will;  then  his  medicine  had  to  be 
taken  and  in  this  way  all  the  time  was  consumed,  till  a  new  round  of 
measures  was  begun  with  renewal  of  the  compress  for  the  neck. 

If  bv  tiie  use  of  the  head  mirror  we  have  tliscovered  redness  and 
swelling  of  the  tympanic  membrane,  we  instill  5  per  cent,  carbolic  acid 
in  glycerin,  lukewarm,  two  or  three  times  a  day,  and  use  hot  or  cold 
compresses  (depending  on  which  is  more  agreeable)  of  aluminum  acetate 
over  the  ear.  If  there  is  pronounced  infection  and  bulging,  paracentesis 
is  performed;  only,  however,  when  there  is  high  fever  and  pain. 

In  our  experience  the  course  of  otitis  is  not  distinctly  influenced 
by  paracentesis.  In  two  cases,  in  which  paracentesis  was  employed 
on  one  side  and  the  other  allowed  to  perforate  spontaneoush^  the  same 
course  followed  in  both  ears.  In  one  case,  both  sides  healctl  simultane- 
ously;  in  the  other,  chronic  otorrhoea  resulted  on  both  sides. 

If  otorrhoea  has  set  in,  we  clean  the  ear  by  the  instillation  of  3  per 
cent,  hydrogen  peroxide  twice  daily. 

Several  drops  of  this  solution  arc  poured  into  the  pinna,  while  the 
child  is  in  the  recumbent  position.  We  wait  until  the  fluid  acciuires 
body  temperature  and  then,  by  raising  the  pinna,  allow  it  to  flow  into 


SCARLET  FEVER  315 

the  meatus.  If  the  secretion  is  purulent,  a  good  deal  of  foam  results,  a 
fact  which  may  also  be  of  diagnostic  value. 

Lymphadenitis. — The  compresses  about  the  neck,  mentioned  in 
connection  with  the  treatment  of  pharyngeal  affections,  also  suffice  for 
mild  lymphadenitis.  If  the  swelling  increases,  we  employ  compresses 
with  1  per  cent,  solution  of  aluminum  acetate,  or  paint  the  surface  with 
5  per  cent,  iodovasogen  (iodine  in  vaseline),  or  cover  it  with  10  per  cent, 
ichthyol  ointment.  A  positive  influence  in  producing  fluctuation  is 
exerted  only  by  freciuently  repeated  hot  poultices  (bread  or  linseed  in  a 
bag).  We  delay  incision  until  distinct  fluctuation  is  present  and  the  skin 
at  the  point  of  fluctuation  is  injected  and  thinner.  The  operation, 
which  is  otherwise  very  painful,  is  performed  with  superficial  ether 
narcosis.  We  warn  against  premature  incision.  If  undertaken  too 
early,  firm  infiltrated  tissue  is  found,  which  shows  an  inclination  to 
secondary  infections. 

The  rlieumatic  complications  are  so  mild  and  transient  that  they  do 
not  require  any  internal  medication;  the  swollen  joints  are  kept  at  rest 
and  wrapped  in  compresses  of  1  per  cent,  acetate  of  aluminium. 

In  severe  cases,  as  we  have  mentioned,  the  heart  is  usuallj-  mark- 
edly afi"ected  (rapid  pulse,  cyanosis,  cold  extremities).  We  employ 
cardiac  stimulants  in  all  such  cases  to  counteract  heart  weakness;  inter- 
nally, digitalis  (infusion  of  leaves,  0.2-0. .5  in  acjuse,  100.0);  black  coffee; 
subcutaneously  alternately  caffeine  (caff,  salic,  1.0;  aq.,  10.0)  and  cam- 
phor (camphor;  1.0;  olei.  oliv.,  10.0);  also  alcohol  (wine  soup,  tea  with 
cognac,  Malaga  wine).  This  stimulating  treatment  in  case  of  heart 
weakness  is  extremely  important.    We  attach  a  great  deal  of  value  to  it. 

Finally,  during  the  entire  course  of  scarlet  fever  the  bowels  nmst 
be  kept  regular.  We  prefer  the  use  of  mild  laxative  waters,  or  castor 
oil  and  manna,  aa.  10.0  (oiiss). 

SERUM    TREATMENT 

The  beneficial  effects  of  diphtheria  antitoxin  have  induced  a  num- 
ber of  authors  to  attempt  a  serum  treatment  of  scarlet  fever.  The  use  of 
the  serum  of  convalescent  patients  (v.  Lcyden)  is  impractical  on  account 
of  the  difficult}^  of  obtaining  it  in  sufficient  quantity.  Besides,  to  judge 
from  its  effects  up  to  the  present  time,  the  serum  has  had  no  certain 
efficiency. 

The  discovery  of  streptococci  in  scarlet  fever  suggested  the  idea  of 
producing  an  active  antistreptococcus  serum  by  immunizing  animals 
against  scarlet  fever. 

Marmorek,  Tavel,  Moser  and  Aronson  all  have  worked  with  this 
end  in  view,  employing  different  methoils.  Except  for  a  few  negative 
experiments  with  Marmorek's  serum  and  ordinary  horse  serum  (see 
Richard  L.,  p.  294),  our  experience  has  been  limited  to  Moser's  serum. 


316  THE   DISEASES   OF   CHILDREN 

\\  c  injected  tlie  latter  into  about  two  hundred  eliildren,  and  are  fully 
convineed  of  its  specificity. 

In  producing  liis  serum,  Moser  worked  with  the  idea  that  scarlet 
fever  streptococci  represent  a  group  with  specific  characteristics.  In 
immunizing  horses,  he  therefore  employed  only  streptococci,  which  had 
been  obtained  in  pure  culture  from  the  heart's  blood  of  patients  who  had 
died  of  scarlet  fe^cr. 

A  second  f(>ature,  in  whi(di  hi.s  method  differs  from  that  of  Mar- 
morek  and  Aronson,  is  that  he  transjilanted  the  streptococcus  on 
bouillon  without  passage  through  animals,  to  avoid  changing  any  of 
the  properties  of  the  organism. 

The  preparation  of  the  serum  differs  from  that  of  diphtheria  anti- 
toxin, in  that  living  bouillon  cultures,  and  not  filtrates,  are  injected 
into  the  horses.  Several  months  later,  the  animals  are  bled  and  the 
separated  serum  caught  sterile  and  filled  into  vessels  without  the  addi- 
tion of  carbolic  acid. 

A  fault  of  the  scarlet  fever  serum,  not  corrected  to  date,  is  the 
large  dosage  required  for  decided  curative  effects.  As  in  the  early  days 
of  diphtheria  antitoxin,  when  even  larger  quantities  of  serum  had  to  be 
administered,  serum  disease  results  nuich  more  frequently  and  intensely 
than  from  the  relatively  small  doses  of  diphtheria  serum  (4-10  c.c), 
which  contain  a  large  amount  of  antitoxin. 

For  this  reason  we  employ  the  scarlet  fever  serum  only  in  the  severe, 
prognosticaUy  douhtjul  cases.  Another  reason  for  avoiding  its  use  in 
mild  cases  is  that  they  recover  without  treatment.  We  are  convinced 
of  the  actual  specificity  of  the  serum,  especially  by  instances  in  which 
we  injected  one  of  two  similarly  affected  members  of  a  family  foi'  ex- 
perimental reasons,   wliile  the  other  was  allowed  to  run  its  course. 

Emilie  St.,  six  years  old,  and  Rosa  St.,  eight  years  old  (Fig.  61), 
took  sick  at  the  same  time  with  malaise,  fever  and  rash.  On  the  follow- 
ing day,  at  10  a.  m.,  they  were  admitted  to  the  hospital.  Each  had  a 
typical  rash,  follicular  tonsillitis,  no  complications.  Temperature  taken 
every  two  hours.  At  9  p.  m.,  Emilie  had  39.9°  C.  (103.8°  F.);  Rosa, 
39.7°  C.  (103. r,°  F.).  The  former  was  injected  with  200  c.c.  of  Moser 
serum.  In  the  first  hours  after  the  injection,  the  temperatures  contin- 
ued alike.  At  1  .\.  m.,  Emilie's  temperature  fell  by  crisis;  temperature 
at  7  A.  M.,  37.2°  C.  (99°  F.),  while  in  Rosa  there  was  a  remission  to  38.7° 
C.  (101.6°  F.),  followed  by  a  rise  to  40.2°  C.  (104.4°  F.).  Emilie's  tem- 
perature in  the  afternoon  rose  only  to  38.1°  C.  (100.6°  F.),  on  the 
following  morning  dropped  to  37.5°  C.  (99.7°  F.),  and  thereafter  was 
normal.  Rosa's  fever  curve  followed  the  ordinary  type  of  scarlet  fever. 
The  temperature  did  not  sink  to  37.5°  C.  (99.7°  F.)  till  the  eleventh 
day.  In  the  chart  only  the  influence  upon  the  temperature  can  be  noted. 
At  the  bedside,  the  action  of  the  serum  is  exhibited  chiefly  in  its  favor- 


SCARLET  FEVER 


317 


able  influence  upon  the  toxic  symptoms  (improvement  of  mental  con- 
dition, of  pulse,  disappearance  of  cyanosis  and  coldness  of  limbs,  cessa- 
tion of  diarrhcea).  Thougli,  judging  from  the  mode  of  its  preparation, 
the  serum  should  be  bactericidal,  and  therefore  might  be  expected  to 
exert  an  influence  on  the  manifest  streptococcus  process,  its  action  is 
really  hke  that  of  an  antitoxic  serum. 

The  action  of  the  serum  is  best  exhibited  in  the  purely  toxic  cases 
(p.  293).  The  best  results  are  obtained  if  the  serum  is  employed  during 
the  first  three  days  of  the  disease.     It  has  an  influence  on   the  infec- 


FiG.  01. 


Effect  of  streptococcus  serum  on  the  temperature. 

tious  phenomena  (pharynx,  nose,  lymph-glands)  only  in  inliibiting  the 
appearance  of  these  processes,  if  injected  early,  and  thus  preserving 
life.  An  effect  on  the  temperature  is  frequently  not  observed.  If 
used  after  the  fifth  day,  the  serum  loses  in  efficiency,  especially  in  the 
severe  infectious  forms.  The  appearance  of  sequela?,  especially  of 
nephritis,  is  not  prevented  by  the  serum. 

The  serum  should  be  employed  only  once  and  at  one  point;  200  c.c. 


318  THE    DISEASES   OF   CHILDREN 

arc  injected  under  the  skin  of  tlic  abdomen  and  the  point  of  puncture 
sealed  with  collodion  and  cotton. 

Naturally,  the  general  (stimulants)  and  local  treatment  (care  of 
mouth,  poultices  about  neck,  etc.)  should  not  be  neglected  on  account 
of  the  serum-therapy. 

Nephritis. — Mild  forms  of  nephritis  recover  without  treatment. 
We  employed  no  therapeutic  measures,  not  even  diaphoresis,  in  a  large 
number  of  cases,  and  became  convinced  that  oedema  and  albumin  dis- 
appeared just  as  rapidly  as  if  packs  and  internal  medication  are  used. 
Other  cases  were  protracted,  whether  treated  or  untreated;  we  were 
impressed  with  the  fact  that  the  course  of  nephritis  is  not  influenced 
by  any  known  mode  of  treatment. 

Rest  in  bed  is  certainly  essential,  since,  if  the  patient  get  up  too 
early,  an  increase  in  the  amount  of  albumin  is  frequently  noted.  We 
also  keep  the  children  on  a  meat-free  diet,  as  long  as  the  albuminuria 
persists;  we  deviate  from  this  rule  only  in  very  protracted  cases  or 
when  there  is  intolerance  of  a  milk  diet. 

Diaphoresis  is  not  absolutely  essential.  To  produce  sweating  we 
employ  no  internal  medication,  but  moist  or  dry  warm  packs,  depend- 
ing on  which  is  more  agreeable  to  the  patient.  If  the  child  becomes 
excited  in  the  packs,  we  use  them  for  only  a  short  time  or  omit  them 
altogether.  We  do  not  restrict  the  ingestion  of  liquids  (milk,  tea,  water, 
alkahne  waters),  but  permit  the  patients  to  drink  when  thirsty. 

We  have  had  too  little  experience  with  a  diet  in  which  common 
salt  is  reduced  in  cpiantity  to  pass  judgment  on  its  value  in  acute 
nephritis.  This  dietetic  measure  is  easily  practiced:  The  food  consists 
of  milk,  cereals,  malt-cofTee,  fruit,  stewed  fruit,  unsalted  bread  and 
butter,  and  honey. 

The  complications  of  nephritis  are  treated  symptomatically.  In 
heart  weakness,  especially,  cardiac  stimulants  are  indicated. 

One  feature  of  severe  nephritis  requires  special  mention:  Urwmia. 
The  treatment  should  be  immediate,  since  delay  may  lead  to  serious 
consequences. 

As  soon  as  the  convulsions  are  pronounced  and  coma  begins,  or  if, 
as  in  the  asthmatic  type,  symptoms  of  pulmonary  ccdema  appear 
(pronounced  cyanosis,  foamy  sputum),  we  resort  to  an  energetic  vene- 
section. This  measure  is  sometimes  executed  with  difficulty  on  account 
of  the  smallness  of  the  brachial  veins  in  children.  We  follow  the  vene- 
section with  a  subcutaneous  infusion  of  200  to  300  c.c.  of  physiological 
salt  solution,  or  high  irrigations  of  lukewarm  water.  Hot  packs  should 
be  avoided  in  uramiia. 

We  have  given  up  the  use  of  leeches  on  account  of  the  annoyance 
occasioned  by  a  continuance  of  hemorrhage  and  the  possibility  of 
infecting  the  a-dematous  skin. 


SCARLET  FEVER  319 

The  result  of  venesection  in  uracniia  is  usually  very  striking.  (See 
case  of  Robert  M.,  p.  299.) 

Gabriele  C,  ten  years  old.  Moderate  scarlet  fever.  On  twent}'- 
first  day  of  disease,  nephritis.  On  twenty-fourth  day,  headache,  lassi- 
tude and  restlessness,  followed  by  a  brief  uneniic  attack.  On  the 
follo\\'ing  morning  by  a  more  severe  attack.  Coma.  Venesection. 
Removal  of  300  c.c.  of  dark  blood.  Then  infusion  of  200  c.c.  salt  solu- 
tion into  right  abdominal  wall.  The  convulsions  decreased  in  inten.ciity 
even  during  the  venesection.  An  hour  later,  the  patient  was  quiet, 
slept  for  two  hours,  and,  on  waking,  was  perfectly  clear  mentally. 

If  the  convulsions  reappear  after  a  time,  venesection  must  be 
repeated. 

Rosa  M.,  seven  years  old.  Venesection  had  to  be  resorted  to  three 
times.  It  is  true  that  the  first  time  only  80  c.c,  the  second  only  100  c.c, 
were  withdrawn.  On  the  third  occasion,  300  c.c.  were  taken.  Improve- 
ment after  each  venesection,  but  the  convulsions  did  not  cease  entirely 
until  after  the  last. 

Venesection,  though,  is  not  always  effective. 

Johanna  T.,  nine  years  old.  Brought  to  hospital  during  ursemic  at- 
tack, and  died  four  hours  later,  after  a  thorough  venesection  (250  c.c), 
in  spite  of  the  simultaneous  administration  of  camphor  and  caffeine. 

Prophylactic  Measures    Against    Nephritis    Have 
Been    Unavailing    Hitherto 

Nephritis  may  appear  in  spite  of  great  precautions  against  catching 
cold  and  careful  supervision  of  diet.  The  prophylactic  drugs  (oleum 
terebinthiniP,  urotropin)  recently  suggested  have  been  entirely  inef- 
fective in  our  hands. 

When  we  stated  that  every  child  should  be  kept  in  bed  for  four 
weeks,  we  referred  only  to  uncomplicated  cases.  Whenever  there  is 
evidence  of  a  sequela,  rest  in  bed  must  naturally  be  correspondingly 
increased.  In  the  case  of  nephritis,  we  do  not  permit  the  children  to 
sit  up  until  at  least  one  week  after  the  last  appearance  of  fever  or  al- 
buminuria. Then  we  increase  the  diet  and  continue  to  take  the  temper- 
ature mornings  and  evenings,  and  to  examine  the  urine.  Several  baths 
are  given  during  this  period.  One  to  two  weeks  after  getting  up,  the 
children  are  allowed  to  go  into  the  open  air. 

Though  scarlet  fever  is  very  deceptive  in  its  course,  it  has  one  ad- 
vantage over  measles,  wliich  is  apparently  so  harmless.  It  does  not 
prepare  the  soil  for  chronic  infections,  and  has  absolutely  no  relation 
to  tuberculosis.  Children,  who  do  not  die  of  the  disease,  in  the  large 
majority  of  cases  do  not  suffer  any  permanent  injury.  Eight  weeks 
after  the  onset  of  the  disease  they  are  as  well  as  ever. 

The  only  persistent   damage,   wliich   occurs  relatively  frequently, 


320  THE   DISEASES   OF   CHILDREN 

is  clironic  otorrhoea  with  resultant  deafness.    More  uncommon  affections 
arc  fuiictiiinal  impairments  due  to  injury  of  the  heart  valves  or  kidneys. 

Simultaneous  Occurrence  of  Several  Acute  Exanthemata. — The 
view  of  Hebra,  that  tlie  acute  exanthemata  mutually  exclude  one 
another,  has  been  recognized  as  untrue  for  forty  years,  as  a  result  of  a 
large  number  of  observations.  All  sorts  of  combinations  occur  between 
scarlet  fever,  chicken-pox,  measles  and  vaccinia.  These  combinations 
are  of  cUnical  interest  only  when  the  rashes  appear  simultaneously. 

Varicella  and  Scarlet  Fever. — The  diagnosis  of  varicella  with 
scarlet  fever  docs  not  present  any  difficulties. 

In  two  such  cases  mc  saw  the  primary  lesion  of  the  scarlatinal 
infection  apparently  occur  in  a  varicella  vesicle — by  secondary  infec- 
tion (Heubncr).     In  one  case  both  rashes  could  be  seen  together. 

Measles  and  Scarlet  Fever. — It  is  much  more  difficult  to  analyze 
a  rash  consisting  of  measles  and  scarlet  fever. 

Marie  Sch.,  five  years  old.  Admitted  to  hospital  without  history. 
On  the  first  day  the  face  was  distinctly  reddened  only  about  the  cheeks. 
The  region  about  the  mouth  showed  no  distinct  measle  spots,  but  was 
not  perfectly  pale.  On  the  body  was  a  small  macular,  scarlatiniform 
rash;  on  the  inner  surfaces  of  the  arms,  somewhat  larger  spots,  with 
irregular  interspaces.  Pronounced  conjunctivitis,  coryza,  dry  cough, 
typical  tongue  of  measles,  Koplik  spots.  At  the  same  time,  congestion 
and  swelling  of  the  tonsils. 

On  the  following  day  the  rash  appearcil  more  morbilliform;  gray- 
ish patches  had  formed  on  the  tonsils,  which  later  developed  into  a 
typical  scarlatinal  angina. 

Fever  dropped  by  lysis,  as  in  scarlet  fever,  with  a  simultaneous 
disappearance  of  the  rash;  otitis  media;  later  desquamation  in  large 
lamella;. 

We  should  not  recommend  the  diagnosis  of  a  double  infection 
merely  because  of  a  doubtful  rash,  since  in  measles  some  parts  of  the 
skin  may  show  a  small  macular  erythema;  on  the  other  hand,  as  we 
have  stated  several  times,  larger  morbilliform  efflorescences  are  obse.':'ved 
in  scarlet  fever  (double  exanthema). 

In  the  preceding  case,  scarlet  fever  is  diagnosticated  positively 
from  the  pharyngeal  manifestation  and  the  flaky  desquamation;  measles 
from  the  changes  in  the  mucous  mendjrane  of  the  mouth  (Koplik),  and 
the  catarrh  of  nose  and  larynx. 

It  is  not  yet  possible  to  determine  whether  the  view  of  Pospischill 
is  correct,  viz.,  that  the  eruptive  stage  of  an  exanthematous  fever  is 
not  only  not  opposed,  but  even  favorable  to  the  implantation  of  a  second 
infection. 


ROTHELN— GERMAN  MEASLES— RUBELLA 

BY 

Professor  J.  von  BOKAY,  of  Budapest 

TRANSLATED   BY 

Dr.  JOHN  RUHRAH,  Baltimore,  Md. 


The  term  "Rubella"  may  be  found  in  medical  literature  as  far 
back  as  1492  but  it  was  not  until  the  eighteenth  century  that  it  was 
used  with  any  degree  of  frequency  by  English,  French  and  German 
writers,  and  even  at  that  time  without  indicating  any  very  great  con- 
fidence in  the  existence  of  the  disease.  Even  in  the  first  ten  years  of 
the  nineteenth  century  the  disease  was  regarded  as  a  new  form  of  measles, 
or  on  the  other  hand,  according  to  the  writings  of  J.  P.  Franks,  Hufe- 
land  and  Heim  as  a  special  manifestation  of  scarlet  fever,  whilst  Schon- 
lein  considered  rotheln  as  a  hybrid  of  measles  and  scarlet  fever.  "Wagner, 
in  1834,  published  in  Hufcland's  Journal  the  first  clear  description  of 
rubella  as  a  separate  clinical  entity.  AYagner's  conclusion  did  not, 
however,  find  manj^  believers  and  even  Canstatt,  in  1847,  and  AVunder- 
lich,  in  1854,  denied  the  existence  of  rubella  as  a  disease  distinct  from 
measles.  In  spite  of  the  excellent  work,  especiallj'  of  the  German  physi- 
cians, Faber,  Salzmann,  Thierfelder,  Mettenheimer,  Emminghaus  and 
Thomas,  the  question  of  the  identity  of  rubella  was  an  open  one  until 
1881.  At  this  time  it  was  discussed  at  the  International  Medical  Con- 
gress, in  London,  and  after  long  arguments  bj'  such  distinguished  English 
and  American  physicians  as  Cheadle,  Shuttlcworth,  AA'.  M.  Squire, 
Jacobi,  J.  Lewis  Smith,  and  others,  every  doubt  as  to  the  existence  of 
rubella  was  dispelled  and  rotheln  was  finally  separated  from  measles 
or  English  measles,  and  the  term  measles  or  rotheln  appeared  in  the 
text  books  as  a  distinct  disease.  In  spite  of  this  some,  especiallj'  the 
Hebra-Kaposi  dermatological  school,  held  to  the  older  view  of  Hufe- 
land  or  of  Schonlein,  even  as  late  as  1887,  in  opposition  to  the  important 
group  of  pediatrists. 

The  co7itagionsness  of  rubella  is,  according  to  the  uniform  agree- 
ment of  all  observers,  much  less  than  that  of  measles  and  consequently 
very  wide  spread  epidemics  are  rarely  observed.  The  infection  is  usually 
direct.  It  appears,  however,  that  the  contagious  principle  can  adhere 
to  objects  and  also  to  a  third  person  and  the  disease  may  be  transmitted 
by  either  of  these  means.    The  source  of  the  infection  is  generally  some 

11—21  321 


322  THE    DISEASES   OF   CHILDREN 

place  where  children  are  brought  in  close  contact  with  one  another,  as 
in  schools,  asylums  and  playgrounds.  Most  observers  are  of  the  opinion 
that  infection  takes  place  more  readily  in  closed  rooms  than  in  the  open 
air,  as  in  closed  rooms  the  conditions  for  the  accumulation  of  the  con- 
tagion are  better,  and  many  deny  the  j)ossibility  of  infection  in  the  open 
air  in  ordinary  intercourse.  This  opinion  seems  to  find  support  in  the 
fact  that  infection  is  most  frequent  in  winter  and  in  rainy  weather  when 
the  children  are  compelled  to  remain  indoors  and  when  the  air  of  the 
rooms  is  in  an  unusually  favorable  condition  for  the  transmission  of 
the  disease.  In  closed  rooms  in  institutions  the  number  of  cases  may 
reach  considerable  in  a  very  short  time.  In  Chicago,  in  1881,  in  an 
orphan  asylum  in  a  comparatively  short  time  there  were  95  cases;  in 
a  New  York  Deaf  and  Dumb  Asylum,  in  1883,  out  of  450  inmates,  95 
were  infected  and  according  to  the  report  of  Hatfield  there  were  110 
cases  out  of  196  children  in  an  asylum.  The  epidemics  last  according 
to  my  own  personal  experience  from  2  to  4  months. 

An  (iltack  of  Rotheln  does  not  protect  from  an  attack  of  measles  or 
scarlet  fever  nor  does  an  attack  of  measles  or  scarlet  fever  furnish  any 
imvnmity  from  Rotheln.  This  important  fact  has  been  established  by 
thirty  years  of  careful  observation  and  numerous  examples,  and  it 
can  be  stated  that  rubella  is  a  specific  disease  which  is  to  be  sepa- 
rated from  the  other  acute  infections  and  especially  from  measles  and 
scarlet  fever. 

Recently  (1902)  Vitline  has  reported  an  instance  in  "Wratch''  in 
which  during  a  short  period  the  same  patient  had  measles,  rotheln 
and  scarlet  fever.  The  greatest  susceptibility  to  infection  is  in  children 
from  two  to  ten  years  of  age.  Nurslings  are  seldom  affected,  SchoU 
saw  a  case  of  intra-uterine  infection  when  the  eruption  developed  a 
few  days  after  birth.  Edwards  has  noted  the  occurrence  of  true  rubella 
in  the  adult  and  Seitz  has  observed  a  case  of  the  disease  in  a  woman  of 
seventy-three.     I  have  noted  it  repeatedly  in  the  adult. 

The  contagiousness  reaches  its  greatest  at  the  height  of  the  eruption 
and  it  is  my  own  experience  and  that  of  others  that  at  the  beginning 
of  the  eruption  and  also  when  it  is  fading  from  the  skin  there  is  little 
or  no  danger  of  spreading  the  disease.  According  to  Thierfelder  the 
contagiousness  is  greatest  whilst  the  eruption  is  fading.  Owing  to  the 
short  period  of  efflorescence  the  danger  of  infection  is  much  less  in 
rubella  than  in  scarlet  fever  or  measles,  the  average  duration  of  the 
eruption  being  3  or  4  days.  The  nature  of  the  contagious  principle  is 
at  present  unknown.  Edwards  found  a  micrococcus  in  the  blood  of 
rubella  patients  and  this  he  believed  to  be  the  cause  of  the  disease  but 
later  in  1890  he  withdrew  his  claims  for  it. 

The  incubation  period  is  on  an  average  of  14  days  according  to 
the  observations  of  Thierfelder  and  Mettenheimer,  whilst  according  to 


GERMAiN  MEASLES  323 

Thomas  and  Emminghaus  it  is  from  15  to  20  days.  It  is  certainly  true 
that  the  incubation  period  of  rubella  is  an  uncommonly  long  one  and 
indeed  longer  than  that  of  varicella.  The  patients  do  not  have  any 
symptoms  during  the  incubation  period.  Plantegna  in  his  report,  in 
1903,  noted  that  in  the  incubation  period  of  rubella  as  in  measles  there 
was  a  leucocytosis  which  changed  to  a  hypoleucocytossis  on  the  appear- 
ance of   the  eruption. 

A  so-called  prodromal  stage  is  either  not  observed  or  lasts  but  a 
few  hours,  rarely  2  or  3  days. 

Forchheimer  has  described  from  observations  in  liis  own  family  a 
prodromal  symptom  consisting  of  a  faintly  marked  pin-point,  rose-red 
enanthem  on  the  soft  palate  which  had  been  noted  previou.sly  by  Em- 
nunghaus,  Thomas  and  Kassowitz.  It  is  important  to  note  that  the 
valuable  diagnostic  sign  of  measles,  Kophk  spots,  have  not  thus  far  been 
noted  (except  by  Widowitz)  either  in  the  prodromal  or  efflorescent 
stages  of  rubella.  The.  older  ^VTiters  describe  a  mild  catarrh  of  the  con- 
junctiva? and  of  the  nasal  mucous  membranes  as  a  prodromal  symptom 
but  this  is  rather  to  be  regarded  as  the  preceding  enanthem  period  of 
the   exanthematous   stage. 

According  to  Theodors,  the  enlargement  of  the  cer\acal  and  occip- 
ital lymph-nodes,  so  characteristic  of  the  stage  of  efflorescence,  may 
sometimes  be  seen  in  the  prodromal  stage.  Koplik  has  also  noted  this 
so-called  prodromal  sign  of  Theodors.  As  a  rule  there  is  no  fever  in  the 
prodromal  stage  and  should  there  be  anj'  elevation  of  temperature  it 
does  not  exceed  38.5°  C.  (101.3°  F.).  In  the  United  States  in  exceptional 
cases  there  have  been  several  days  of  more  or  less  severe  prodromes. 

Symptoms. — The  disease  is  usually  ushered  in  by  the  appearance  of 
the  eruption  and  by  the  above-noted  symptoms.  The  eruption  is  some- 
what hke  that  of  measles  and  hence  the  time  honored  name  "Rubella 
morbillosa."  The  eruption  generally  begins  on  the  head  and  often  on 
the  bridge  of  the  nose  and  the  upper  hps,  and  from  these  parts  it  spreads 
downward  very  rapidly,  reacliing  the  buttocks  in  a  few  hours.  It  is 
an  important  diagnostic  point  that  in  rubella  the  eruption  is  seen  on 
the  hairy  portion  of  the  head  whilst  in  measles  the  scalp  usually  is  not 
affected.  On  the  extremities,  the  eruption  is  principally  on  the  flexor 
surfaces  and  the  palms  of  the  hands  and  the  soles  of  the  feet  are  also 
covered.  In  the  beginning  the  eruption  consists  of  small,  point-like, 
discrete,  slightly  elevated  papules  wliich  soon  change  into  oval  spots 
about  the  size  of  a  lentil,  pale  rose-red  in  color,  tolerably  sharply  out- 
lined, scarcely  raised  above  the  surface  and  lying  rather  close  together. 
In  other  cases  the  rotheln  spots  are  irregular  with  illy  defined  edges 
joined  by  narrow  strips  of  redness  wliich  gives  the  skin  a  marbled  ap- 
pearance. It  is  important  to  note  what  Kophk  mentions  in  liis  article 
in  1900,  that  the  rotheln  spots  do  not  become  confluent,  in  contradis- 


3'ii  THE   DISEASES   OF   CHILDREN 

tinction  to  the  measles  eruption.  Koplik  has  also  called  attention  to 
the  fact  that  the  eruption  has  often  a  scythe-shaped  or  crescent-shaped 
arrangement.  In  cases  where  the  eruption  is  smaller  than  a  lentil  and 
the  spots  very  close  together  it  may  resemble  scarlet  fever.  These  cases 
in  which  the  cliaracteristic  spots  are  not  prominent  and  the  pale  red 
punctate  erujition  predominates  have  been  called  b}'  the  older  writers 
"Rubella  scai'hitinosa."  These  cases,  as  I  shall  explain  in  the  consider- 
ation of  the  "Fourth  Disease,"  should  be  separated  from  tlie  others. 
(See  foot  note  under  Fourth  Disease.) 

It  is  rare  that  one  sees  a  rubella  patient  with  all  of  the  symptoms 
in  the  same  stage.  As  Trousseau  pointed  out,  it  fades  rapidly  when  it 
is  fully  developed  and  so  one  sees  the  eruption  fading  from  one  part 
of  the  body  and  fully  developed  upon  another.  This  rapid  fading  of 
the  rash  is  characteristic  of  rubella  and  furnishes  an  important  differen- 
tial point  in  the  diagnosis  between  this  disease  and  measles.  The  stage 
of  efflorescence  lasts  scarcely  two  or  three  days.  The  fading  of  the 
rash  is  followed  by  a  rapid  disappearance,  leaving  beliind  but  a  tran- 
sient pale  brownish  discoloration.  The  desquamation  is  trifling.  The 
temperature  is  elevated  during  the  eruption  period  but  only  moderately 
and  it  is  of  short  duration.  It  may  rarely  happen  that  the  fever  is  un- 
usually high  (Case  of  Dupres).  At  the  beginning  of  the  eruption  the 
fever  is  at  its  highest  point  or  the  highest  point  may  have  been  reached 
before  the  appearance  of  the  eruption  and  when  the  rash  is  fully  devel- 
oped the  temperature  usually  falls  and  the  patient  is  fever-free.  The 
enlargement  of  the  cerA-ical  lymph-nodes  is  usually  noticeable  during 
the  entire  course  of  the  disease.  Klaatsch  considers  of  especial  impor- 
tance the  enlargement  of  the  postauricular  lymph-nodes  situated  on  the 
upper  part  of  the  mastoid  process  and  I  have  been  able  to  confirm  this 
in  a  number  of  epidemics.  In  describing  several  epidemics  Klaatsch 
says  of  this:  "Tliis  symptom  was  so  constant  in  the  last  epidemics 
that  one  could  make  a  diagnosis  in  the  dark  by  means  of  the  sense  of 
touch  p^o^^ded  he  knew  there  had  been  an  acute  infectious  exanthem 
present."  Here  and  there  swelHng  of  the  lymph-nodes  at  the  angle  of 
the  jaw  occurs  as  well  as  of  the  chain  of  nodes  in  the  back  of  the  neck. 
Musset  noted  in  some  cases  swelhng  of  the  axillary  and  inguinal  nodes. 

During  the  entire  course  of  the  disease  the  general  condition  is  but 
little  affected  and  great  prostration  is  not  observed.  The  patient  coughs 
but  little  and  the  cough  has  a  tracheal  character. 

Various  complications  have  been  noted  and  of  the  most  important 
are  marked  inflammation  of  the  pharynx,  broncliitis  and  broncho- 
pneumonia, acute  catarrh  of  the  stomach  and  bowels,  and  even  choleri- 
form  enteritis,  and  less  often  multiple  serous  inflammation  of  the  joints 
and  painful  swelhng  of  the  thyroid.  Of  these  comphcations  almost 
all  have  been  reported  by  physicians  in  the  United  States.     I  do  not 


GERMAN  MEASLES  325 

remember  having  seen  any  of  them  and  it  seems  that  the  epidemics  of 
middle  Europe  are  Hghter  in  character  than  those  observed  in  America. 

Rubella  vesiculosa  is  a  rare  form  and  analogous  to  niorbilli  vesic- 
ulosi.  Koplik  has  noted  the  occurrence  of  an  abortive  form  in  which 
according  to  his  experience  there  was  notliing  except  the  swelhng  of 
the  cervical  h'mph-nodes.  He  noted  in  numerous  instances  such  swell- 
ing in  individuals  who  had  been  exposed  to  rubella  infections  and  there 
was  subsequently  no  eruption.  In  these  cases  the  swelUng  subsided 
in  a  few  days  mthout  anj-  febrile  disturbance.  I  .shall  not  consider 
Tschamer's  so-called  "ortUchen  Rotheln"  as  I  am  of  the  opinion  that 
it  has  notliing  to  do  with  the  cUnical  feature  of  rubella. 

In  sporadic  cases  the  diagnosis  of  rubella  is  difficult,  as  it  resembles 
mild  cases  of  measles  as  well  as  the  the  abortive  forms.  In  epidemics 
the  diagnosis  presents  no  special  difficulties  and  it  maj'  be  separated 
from  measles  by  the  milder  course,  the  sHght  febrile  disturbance  and 
the  absence  or  mildness  of  the  catarrhal  symptoms.  The  swelling  of 
the  cervical  lymph-nodes,  and  the  so-called  Theodors'  prodomal  sign 
are  of  value  and  particularly  the  absence  of  Koplik  spots.  If  the  disease 
follows  a  measles  epidemic  and  the  patient  has  had  measles  a  short  time 
before  the  diagnosis  presents  but  little  difficulty.  Schmidt  has  noted 
that  the  urine  in  rubella  does  not  give  the  diazo  reaction  whilst  in 
measles  this  reaction  is  present.  It  is  interesting  to  note  that  antitoxin 
rashes  have  been  mistaken  for  German  measles. 

The  course  of  the  disease  is  so  mild  that  the  physician  has  but  little 
to  do  and  the  saying  "Vix  nomen  morbi  merebatur"  applies.  The 
child  does  not  feel  ill  as  a  rule  and  the  patient,  if  an  adult,  can  scarcely 
believe  that  he  is  suffering  from  an  infectious  disease. 

In  most  instances  the  physician  is  called  only  to  give  advice  because 
the  parents  are  afraid  that  the  disease  is  either  scarlet  fever  or  measles. 

In  the  United  States  epidemics  of  a  severe  character  have  been 
described  and  the  mortality  rate  has  been  as  high  as  from  4-9  per  cent. 
It  is  possible  that  there  may  have  been  an  error  in  diagnosis  in  these 
fatal  cases. 

There  are  no  especial  instructions  for  treatment  in  rubella.  Owing 
to  the  mild  character  of  the  disease  isolation  is  rarely  advisable.  Ashby 
recommends  isolation  for  3-4  weeks  which  is  too  long  when  the  short 
duration  of  the  infectious  period  is  considered.  In  Germany  the  sanitary 
regulations  for  rubella  are  the  same  as  for  measles.  The  imperial  Russian 
sanitary  code  forbids  children  who  have  been  in  contact  with  rubella 
patients  to  attend  school  for  IG  da}'s  and  the  patient  must  not  attend 
school  until  two  weeks  after  the  beginning  of  the  exanthem. 


DUKES'  "FOURTH  DISEASE" 

BY 

Professor  J.  von   BOKAY,  of  Bbdapest* 

TRANSLATED    BY 

Dr.  JOHN  RUHRAH,  Baltimore,  Md. 


The  term  "Fourth  Disease"  is  first  found  in  the  Lancet  of  July  14, 
1900,  in  an  article  by  Dr.  Clement  Dukes,  head  physician  to  the  Rugby 
School,  entitled  "On  the  Confusion  of  Two  Different  Diseases  under 
the  Name  Rubella. "  Since  then  the  term  has  been  in  use  chiefly  by 
English  physicians. 

Dukes'  "Fourth  Disease"  is  a  mild  affection  like  rubella  but  with 
a  rash  resembling  scarlet  fever  as  the  rubella  rash  resembles  measles, 
or  we  might  say  that  it  resembles  closely  the  abortive  form  of  scarlet 
fever. 

One  naturally  asks  upon  what  observations  does  Dukes  base  his 
opinion  that  the  "Fourth  Disease"  exists  as  a  separate  clinical  entity. 
In  1892,  Dukes  was  asked  advice  concerning  sixteen  cases  supposed  to  be 
scarlet  fever.  These  cases  occurred  in  a  public  school  and  Dukes,  hav- 
ing been  convinced  that  a  "Fourth  Disease"  existed,  and  believing  that 
the  cases  in  question  were  of  this  nature,  isolated  the  patients  for  only 
fourteen  daj's.  Notwithstanding  the  short  period  of  isolation  there  were 
no  infections  in  the  families  of  the  children.  Dukes  relates  another  epi- 
demic also  in  a  school  where  thirty-one  typical  scarlet  fever  cases  were 
mixed  with  cases  of  the  "Fourth  Disease."  The  incubation  period  of 
the  latter  was  from  fourteen  to  fifteen  days  whilst  that  of  the  scarlet 
fever  cases  was  but  two  or  three  days.  In  nine  cases  the  patients  had 
first  the  "Fourth  Disease"  and  then  scarlet  fever  and  one  patient  had 
scarlet  fever  first  and  the  "Fourth  Disease"  later.  There  were  two 
fatal  cases  of  scarlet  fever  in  this  epidemic.  Dukes  also  observed  an- 
other pupil  who  had  previously  had  scarlet  fever  and  then  the  "Pourth 
Disease."  Many  of  the  patients  who  had  the  "Fourth  Disease"  had 
previously  been  attacked  by  rubella.  In  a  third  house-epidemic  there 
were  nineteen  cases  of  "Fourth  Disease"  and  42  per  cent,  of  these 
patients  had  previously  had  rubella. 


♦This  chapter  has  been  translated  and  allowed  to  remain  as  originally  written.  The  translator  is  of 
the  opinion,  however,  that  whilst  there  may  be  a  fourth  disease  there  has  not  been  sufficient  proof  of  it  and 
he  would  tiierefore  at  least  for  the  present  classify  all  such  cases  as  Rubella.  In  his  experience  one  of  the 
distinctive  features  of  rubella  is  the  polymorphous  character  of  the  eruption,  like  measles  in  one  case,  like 
scarlet  fever  in  another  and  like  a  mixture  of  the  two  in  others. — J.  R. 
326 


DUKES'  "FOURTH  DISEASE"  327 

Owing  to  Dukes'  observations  covering  years  of  experience  the 
author  considers  the  "  Fourth  Disease"  as  a  distinct  affection  quite  in(h'- 
pendcnt  of  measles  and  scarlet  fever.  The  following  account  is  based 
largely  on  Dukes'  publications. 

Except  for  trifling  pain  in  the  throat  the  so-called  'prodromal 
symptoms  are  wanting  in  most  cases,  although  occasionally  there  may 
be  a  chill  and  several  hours  of  nausea,  headache,  backache  and  loss  of 
appetite.  The  incubation  period  varies  from  nine  to  twenty-one  da^'s 
resembling  rvibella  and  differing  markedly  from  scarlet  fever.  The  erup- 
tion is  usually  the  first  indication  of  the  disease  and  it  may  cover  half 
of  the  body  in  a  few  hours.  The  eruption  is  small  and  thickly  set,  pale 
red  and  scarcely  raised  above  the  surface.  This  exanthem  is  also  seen  on 
the  face  but  according  to  Dukes  less  clearly  and  not  at  all  on  the  nose 
or  region  of  the  lips.  The  pharynx  is  somewhat  swollen  and  markedly 
congested.  The  tongue  is  coated  but  the  typical  scarlet  fever  tongue 
is  not  present.  The  lymph-nodes  of  the  neck  are  swollen,  hard  and 
about  the  size  of  a  pea  and  the}'  do  not  attain  the  size  of  the  nodes  in 
rubella.  In  some  cases  the  axillary  and  inguinal  nodes  are  enlarged. 
The  eruption  fades  quickly  and  is  followed  by  a  mild  but  recognizable 
desquamation  which  is  complete  in  about  two  weeks.  Exceptionalh' 
the  desquamation  may  be  very  marked.  Nephritis  is  a  rare  sequel; 
but  a  trifling,  rapidly  disappearing  albuminuria  may  be  observed. 

There  are  few  general  symptoms  and  the  pulse  rate  is  unaffected 
in  the  mild  cases  whilst  it  varies  with  the  temperature  in  the  more  severe 
ones.  The  temperature  ranges  from  37°  C.  to  40°  C.  (98.4°  F.  to  104°  F.). 
Any  symptoms  that  are  present  disappear  when  the  rash  fades.  The 
infectiousness  is  trifling  at  the  onset  and  disappears  entirely  in  two  or 
three  weeks.  The  patient  is  ready  to  get  out  of  bed  in  15  or  16  days; 
the  isolation  may  be  ended  in  two  or  three  weeks. 

I  have  described  the  "Fourth  Disease"  according  to  the  author's 
account  of  it  and  noted  how  closely  it  resembles  abortive  scarlet  fever. 
But,  as  we  have  seen,  the  characteristics  of  the  '"Fourth  Disease"  are 
its  mild  course,  the  absence  of  complications  and  sequela-,  the  rapid 
disappearance  of  the  infectiousness  and,  what  I  consider  of  especial 
importance,  the  long  incubation   period. 

Dukes'  article  started  a  rather  lively  discussion  amongst  English 
and  American  authors  and  whilst  part  of  them  (W.  H.  Broadbent,  Th. 
Johnstone,  J.  J.  "Weaver,  A.  Croick,  A.  L.  Millard  and  Walter  Kidd) 
agreed  with  Dukes,  others  (C.  K.  Millard,  A.  Rutter,  F.  F.  Caiger,  F.  J. 
Poynton,  William  Watson,  J.  W.  Washlnirn,  Ker,  F.  C.  Curtis,  H.  L.  K. 
Shaw)  thought  Dukes'  conclusions  erroneous  and  that  cases  of  the 
"Fourth  Disease"  should  be  classed  as  either  scarlet  fever  or  rubella. 

The  article  of  ,7.  J.  Weaver  furnishes  the  most  conclusive  evidence. 
His  experience  was  as  follows:     Some  months  prior  to  Dukes'  publi- 


328  THE    DISEASES   OF   CHILDREN 

cation,  he  noted  in  tlie  Soutiiport  Borough  Infectious  Disease  Hospital 
in  which  he  was  medical  superintendent,  in  a  number  of  scarlet  fever 
cases,  recurrences  with  a  now  eruption  and  fever.  In  20  cases  of  scarlet 
fever  6  such  recurrences  were  noted  in  three  months.  He  reported  14 
hospital  cases  in  his  experience  with  their  temperature  charts.  These 
charts  are  of  especial  interest  because  they  are  in  cases  in  which  the 
"Fourth   Disease"  either  preceded  or  followed  scarlet  fever. 

The  clinical  picture  of  Weaver  agreed  in  the  main  with  that  of 
Dukes.  He  called  attention  to  the  regular  fine,  punctiforni  character 
of  the  eruption  and  noted  that  in  his  cases  the  rash  appeared  first  on 
the  face  and,  contrary  to  scarlet  fever,  involved  the  skin  surrounding 
the  nioutli.  Certain  rather  negative  features  ho  considers  characteristic 
of  the  disease,  little  or  no  fever,  little  or  no  disturbance  of  the  pulse, 
very  slight  pharyngitis  and  practically  no  general  symptoms.  There 
was  no  strawberry  tongue,  and  the  incubation  was  nine  to  twenty-one 
days.  The  mildness  of  the  symptoms  of  course  suggests  rubella  but 
neither  coryza  nor  cough  was  observed,  and  the  swelling  of  the  cervical 
lymph-nodes  was  less  marked  and  not  so  constant  as  in  rubella  and 
lastly,  in  his  cases  there  was  no  marked  desquamation  but  a  simple 
scaly  separation  of  short  duration. 

The  existence  of  the  "  Fourth  Disease"  as  a  separate  clinical  entity 
can  only  be  determined  by  a  series  of  unprejudiced  observations  but 
one  can  state  that  there  exist  mild  epidemics  suggestive  of  scarlet  fever 
which  attack  children  who  have  already  had  scarlet  fever  and  rubella 
and  it  does  not  protect  the  patient  from  a  subsequent  attack  of  either 
scarlet  fever  or  ruljella. 

All  of  us  who  have  to  do  with  the  acute  exanthemata  either  in  the 
hospital  or  in  private  practice  have  doubtless  seen  such  cases  as  Dukes 
and  Weaver  have  described.  I  myself  have  repeatedly  seen  such  cases 
but  unfortunately  have  not  made  such  observations  as  would  serve  to 
clear  up  definitely  tliis  question.  It  must  be  noted,  however,  that  the 
observations  of  Dukes  and  Weaver,  however  convincing  they  may  be, 
do  not  suffice  to  solve  the  interesting  and  important  question.  When 
we  search  the  hterature  of  rubella  we  find  much  which  in  my  opinion 
goes  to  show  that  Dukes  is  on  the  right  track  and  that  his  opinions  will 
be  verified. 

It  is  remarkable  that  Dukes,  before  he  pubhshed  his  important 
studies,  did  not  search  the  foreign  literature  for  there  he  would  have 
found  much  enhghtenment  upon  tliis  subject.  In  1885  Nil  Filatow, 
in  an  article  in  Russian,  raised  this  question  and,  in  1S96,  in  his  lectures 
on  the  infectious  diseases  of  children  outlines  in  a  special  chapter  a 
separate  disease  similar  to  the  one  which  Dukes  described.  Naturally 
he  did  not  include  under  the  heading  "rubella  scarlatinosa"  those  cases 
of  rubella  in  which  in  addition  to  the  typical  spots  there  is  an  erythem- 


DUKES'  "FOURTH  DISEASE"  329 

ntous  eruption.  According  to  Filatow,  rulaella  scarlatinosa  is  "a  sepa- 
rate acute  infectious  and  contagious  disease,  whicli  is  cliaracterized  by 
a  scarlatiniform  eruption  but  whicli  may  be  separated  from  scarlet 
fever  by  the  mild  course  and  especially  by  the  difference  in  the  con- 
tagiousness." 

In  my  opinion  megalerythema  epidemicuni  or  erythema  infecti- 
osum  (or  the  fifth  disease,  as  the  latest  French  writers  would  call  it) 
which  has  been  a  matter  of  discussion  in  the  German  hterature  since 
1900,  has  notliing  to  do  with  the  "  Fourth  Disease  "  The  disease  des- 
cribed by  Trommer  in  1901  as  scarlatinois,  and  that  which  Pospischil 
called  scarlatinoid  have  no  bearing  on  the  question  of  the  existence 
of  the  fourth  disease. 


VARICELLA 

HY 

Dr.  N.  SWOBODA,  of  Vienna 

translated  by 
Dr.  JOHN  RUHRAH,  Baltimore,  Md. 


Varicella  is  still  described  in  most  of  the  text  books  as  a  disease 
which  is  uniformly  harmless,  of  characteristic  appearance  and  which 
rarely   needs   any   treatment. 

Tn  the  past  two  decades,  however,  a  number  of  interesting  observa- 
tions have  been  made  wliich  show  that  the  ordinary  conception  of 
varicella  is  erroneous  and  that  there  may  be  comphcations  which  threaten 
hfe,  great  variations  from  the  usual  clinical  picture  and,  what  is  of 
especial  importance,  it  may  often  be  confused  with  smallpox.  The 
number  of  these  observations  is  so  great  that  more  than  mere  mention 
of  the  most  important  of  them  is  not  possible  in  the  allotted  space. 

It  is  impossible  to  state  just  how  far  back  the  history  of  varicella 
reaches.  Hesse  (1829)  cites  a  number  of  authors  who  thought  they 
recognized  varicella  in  the  writings  of  the  old  Greek,  Roman  and  Arabian 
physicians  but  they  have  not  been  able  to  present  much  evidence  to 
support  their  views.  However,  in  the  wTitings  of  Vidus  Vidius  (1626) 
and  some  of  liis  contemporaries  (Ingrassius,  Duncan  Liddle)  it  is  plain 
the  clinical  picture  of  varicella  was  well  known  and  was  differentiated 
from  smallpox.  Vidus  named  the  disease  CrystaUi  and  mentions  that 
the  people  call  it  Ravaglione,  a  name  wluch  is  still  used  in  Haly. 

The  history  of  varicella  cannot  be  entered  into.  SufTice  it  to  say 
that  during  the  next  two  centuries  many  authors  wrote  upon  the  sub- 
ject (see  Hesse  for  citations)  some  claiming  and  others  disclaiming  its 
identity  with  smallpox. 

Amongst  those  who  recognized  and  described  the  disease  may  be 
mentioned  Heberden  (1767),  Willan  (1808),  Heim  (1809)  and  Thom- 
son (1820,  21,  22).  Hesse  (1829)  has  pubhshed  the  most  complete 
monograph  upon  the  subject. 

The  disease  remained  unknown  by  the  great  mass  of  practitioners 
and  it  was  not  until  the  introduction  of  inoculation  and  vaccination 
that  the  subject  became  one  of  general  interest. 

In  the  first  decade  of  the  nineteenth  century,  smallpox  was  noted  in 
vaccinated  persons  and  it  also  happened  that  varicella  was  frequently 
mistaken  for  smallpox.      As  the  opponents  of  vaccination  used  this  as 

330 


VARICELLA  331 

an  argument  in  favor  of  the  uselessness  of  the  procedure  this  little  known 
disease,  chicken-pox,  was  carefully  studied  and  separated  from  smallpox. 
The  monographs  of  Willan  (1808)  and  Heim  (1809)  showed  that  varicella 
was  responsible  for  most  of  the  so-called  recurrences  after  vaccination. 

In  the  following  decades  the  idea  that  varicella  was  a  separate 
disease  gained  ground  and,  in  Germany  at  least,  by  the  forties  tliis  was 
the  common  opinion  of  physicians. 

About  tliis  time  the  influential  "\'iennese  dermatological  school 
under  Hebra  declared  dogmatically  that  varicella  and  variola  were 
identical  and  it  appears  that  physicians  generally  were  converted  to 
this  opinion.  The  great  smallpox  epidemic  of  1870-1873  again  brought 
up  the  question  of  identity  and  a  controversy  was  once  more  begun 
the  vehemence  and  pertinacity  of  wliich  is  scarcely  duphcated  in  medical 
history.  As  a  result  of  this  controversy  most  physicians  have  returned 
to  the  idea  that  varicella  and  smallpox  are  separate  and  distinct  diseases. 

Varicella  originates  only  through  infection,  but  concerning  the 
nature  of  the  contagion  we  know  nothing  and  we  can  only  surmise  as  to 
the  method  of  transmission  and  as  to  its  portal  of  entry  into  the  body. 
It  is  certain  that  infection  occurs  easily  when  a  child  is  brouglit  into 
direct  contact  with  one  suffering  from  the  disease,  or  when  it  i-emains 
in  the  same  room  for  a  short  time.  Infection  through  the  air  seems  to 
play  a  considerable  part  in  the  transmission  of  the  disease.  The  tenacity 
of  the  poison  of  chicken-pox  is  sHght  and  is  practically  disregarded  in 
practice  and  thus  is  just  the  opposite  of  smallpox  in  which  the  infec- 
tious material  may  be  carried  great  distances  and  live  for  almost  in- 
definite periods.  Many  physicians  of  great  experience  doubt  if  varicella 
is  ever  carried  by  a  tliird  person  or  by  fo mites. 

The  infectiousness  begins  with  the  appearance  of  the  eruption 
(Cerf,  1901,  and  Apert,  1895)  and  disappears  even  before  the  last  crusts 
have  separated.  The  susceptibility  to  the  disease  is  very  general,  espe- 
cially during  childhood.  Daily  experience  teaches  that  when  a  child  is 
taken  ill  in  a  family,  closed  institution,  asylum  or  school  the  majority  of 
the  other  children  take  the  disease  even  if  the  child  is  at  once  isolated. 

Whilst  the  susceptibihty  to  the  ordinary  mode  of  infection  is  very 
general,  varicella  can  probably  not  be  transmitted  by  inoculating  healthy, 
susceptible  cliildren;  so  that  the  contents  of  the  varicella  vesicle  and 
variola  pustule  differ  essentially.  At  any  rate  the  inoculation  succeds  only 
exceptionally  under  especially  favoraljle,  and  to  us  unknown,  conditions. 

Numerous  inoculation  experiments  were  tried  during  the  first  half 
of  the  last  century  partly  for  purposes  of  chfferential  diagnosis  and 
partly  to  demonstrate  the  difference  between  variola  and  varicella. 
(For  literature  until  1829  see  Hesse).  The  results  were  generally  nega- 
tive and  the  exceptional  successes  consisted  in  a  generalized  exanthem 
and  not  in  a  localized  vesicle  at  the  site  of  inoculation. 


332  THE    DISEASES   OF   CHILDREN 

It  is  certain  in  all  inoculation  experiments  that  the  contents  of  the 
varicella  vesicles  cannot  cause  smallpox  either  in  vaccinated  or  un- 
vaccinated  individuals 

One  attack  usually  confers  a  lasting  immunity  and  exceptions  are 
exceedingly  rare.  Just  as  there  are  individuals  who  obtain  an  unusually 
high  grade  of  immunity  through  an  attack  of  varicella  with  marked 
intoxication  symptoms,  so  on  the  other  hand  there  are  those  who  get 
but  a  shght  inniiunity  from  a  very  mild  attack  and  may,  therefore, 
have  a  second  attack  These  are  hardly  to  be  considered  under  the 
ordinary  rule,  however,  as  the  second  attack  follows  closely  upon  the 
first.  In  the  older  Hterature  instances  are  found  in  the  writings  of  Helm, 
Hiifeland,  Canstatt  and  Trousseau  and  in  the  more  recent  pubhcations 
of  Comby,  Blair,  Butler,  Netter,  etc.  The  interval  has  been  as  follows: 
fourteen  days  (Vetter,  1860),  ten  days  (R.  Neale,  1891),  nineteen  to 
twenty-two  days  in  four  cases  (Dawes,  1903).  Kassowitz  saw  a  patient 
who  had  two  severe  attacks  with  an  interval  of  one  and  a  half  years, 
and  Gerhardt  treated  a  child  who  had  three  attacks. 

The  susceptibihty  is  not  influenced  by  the  occurrence  of  other  diseases. 
Varicella  may  be  present  at  the  same  time  as  some  other  disease  or 
may  immediately  precede  or  follow  it.  Varicella  is  easier  to  tell  when 
it  occurs  with  some  other  acute  infection  on  account  of  the  vesicular 
eruption  being  much  more  easily  distinguished  from  measles,  scarlei 
fever  or  rubella  than  these  are  from  one  another. 

In  the  older  literature  there  are  numerous  examples  of  the  occur- 
rence of  one  or  two  infectious  diseases  at  one  time  with  varicella  (le 
Roux,  Reuss,  Boehm,  cited  by  Hesse)  and  in  the  more  recent  times  the 
following  may  be  cited:  Thomas  (1871),  Fleischmann  (1870),  Prior 
(1883),  Lichtmann  (1892),  Szczypiorsky  (1895)  Netter  (1894),  Bery 
(1898),  Hcubner  (1904),  and  others  in  French  and  English  literature 
cited  by  Cerf.  Observations  of  this  kind  are  rare  when  confined  to  pri- 
vate practice  but  in  children's  hospitals  such  occurrences  are  not  at 
all  uncommon. 

The  relation  of  scarlet  fever  to  varicella  is  of  interest  and  the  scarlet 
fever  virus  may  enter  through  a  varicella  pustule.  Heubner  (1903) 
noted  that  when  scarlet  fever  attacked  a  chicken-pox  patient  the  red- 
ness spread  from  a  scratched  pustule  just  as  it  would  from  a  wound. 
Pospischil  (1904)  gathered  from  his  large  material  that  scarlet  fever 
attacked  varicella  patients  particularly  in  the  first  stage  when  the  new 
vesicles  were  making  their  appearance.  He  believes  that  the  majority 
of  general  streptococcus  infections  following  measles  and  varicella  are 
due  to  infection  with  scarlet  fever.  Cerf  (1901)  has  noted  that  nearly 
all  the  varicella  that  follows  scarlet  fever  is  attended  by  suppuration. 

Of  much  more  importance,   however,  is  the  simultaneous  occur- 


VARICELLA  333 

rence  of  varicella,  variola  and  vaccinia,  or  of  the  immediate  sequence 
of  the  same,  because  the  independence  of  varicella  is  thus  noted.  The 
onset  of  varicella  during  or  immediately  after  vaccination  is  of  frequent 
occurrence.  It  may  be  noted  at  the  time  of  vaccination.  Varicella 
may  appear  at  the  same  time  as  variola.  Whilst  Thomas  (1874)  neither 
saw  nor  believed  in  this,  we  have  nevertheless  a  number  of  unprejudiced 
observations.  Bourland  (1894)  saw  both  diseases  during  a  double  epi- 
demic and  Pages  (1902)  the  simultaneous  occurrence  of  variola,  vari- 
cella and  vaccinia.  J.  F.  Schamberg  (1902)  saw  a  case  of  varicella 
brought  into  a  smallpox  hospital  and  the  disease  developed  in  33  chil- 
dren with  variola.  In  some  cases  only  seventeen  days  elapsed  between 
the  appearance  of  the  two  eruptions.  Where  the  idea  of  the  identity 
of  the  diseases  prevails  and  patients  with  varicella  are  isolated  with 
smallpox  cases,  unless  the  former  have  been  protected  by  vaccination 
they  will  have  an  attack  of  smallpox  (Lothar  Meyer,  Steiner,  Forster, 
Quincke,  Fleischman,  Eisenschitz  and  others). 

Vaccination  takes  in  children  who  have  had  chicken-pox  and  runs 
the  same  course  as  in  those  who  have  not  had  it, — a  fact  which  any 
physician  can  easily  verify,  and  there  are  numerous  references  to  this 
in  the  literature  of  the  last  half  century. 

The  accidental  occurrence  of  varicella  or  variola  during  the  course 
of  the  disease  has  a  practical  significance. 

We  will  now  consider  the  reasons  why  the  two  diseases  are  not 
identical.  As  has  already  been  stated,  some  physicians  believe  that 
the  two  diseases  are  only  differences  in  intensity  of  a  single  disease. 
We  have  not  room  to  consider  in  detail  the  century  long  discussion  but 
will  give  only  the  important  facts  which  show  that  the  view  of  the 
dualists  is  correct.  One  should  remember  that  in  many  of  the  discus- 
sions the  views  of  the  dualists  were  not  always  correct  and  some  of  their 
claims  were  not  based  upon  sound  observations. 

1.  Inoculation  with  the  contents  of  the  varicella  vesicle  always 
procUices   varicella   and  never  variola. 

2.  The  occurrence  of  varicella  does  not  protect  from  variola  or 
vaccinia  and  the  reverse  is  also  true. 

3.  The  third  question  which  has  been  discussed  at  great  length  is 
whether  a  patient  with  varicella  can  cause  variola  in  another  and  this 
may  be  answered  in  the  negative. 

^'aricella  is  a  disease  etiologically  different  from  variola  but  which 
at  times  has  clinical  manifestations  greatly  resembling  smallpox. 

OCCURRENCE;  MODE  OF  SPREADING;  AGE  INCIDENCE 

Varicella  is  a  disease  which  occurs  among  all  races  and  which  never 
disappears  entirely  from  the  larger  cities.  Large  and  small  epidemics 
arc  of  frequent  occurrence  and  are  most  often  seen  about  the  time  of 


334  THE   DISEASES   OF   CHILDREN 

the  opening  of  the  schools.  Nearly  all  the  epidemics  aie  of  a  benign 
character  but  exceptionally  there  may  be  numerous  cases  of  nephritis, 
secondary  infections,  or  gangrene.  Unusually  wide  spread  epidemics 
have  occurred,  however,  in  which  the  disease  resembles  variola  in  its 
course,  the  epidemic  described  by  Mombcrt  occurring  at  Kurhesscn  in 
1824  may  be  cited  as  an  example. 

Varicella  is  almost  exclusively  a  disease  of  childhood  and  some 
authors.  Senator  for  example,  have  gone  so  far  as  to  speak  of  an  immunity 
in  adults,  and  have  given  this  as  a  point  in  differential  diagnosis.  Others 
state  that  the  disease  is  of  such  exceptional  occurrence  in  adults  that 
all  cases  occurring  in  grown  people  should  be  under  the  supervision  of 
the  sanitary  authorities.  On  this  account  adult  patients  with  varicella 
have  been  sent  to  smallpox  hospitals  and  have  there  contracted  variola. 
During  the  past  year  there  have  been  such  a  large  number  of  cases  in 
grown  people  in  places  which  were  previously  and  have  remained  free 
from  smallpox  that  the  question  of  the  occurrence  of  chicken-pox  in 
later  life  may  be  regarded  as  settled. 

However  this  may  be,  every  case  of  chicken-pox  in  an  adult  should 
be  gone  into  carefully  to  avoid  the  possibilities  of  error.  It  is  especially 
important  to  remember  that  a  variola-like  exanthem  is  common  in  the 
varicella  of  adults.  Doubtful  cases  should  be  handled  in  the  same  way 
as  smallpox  owing  to  the  probability  of  its  being  that  disease  and  the 
danger  of  spreading  the  contagion  if  it  should  be. 

The  incubation  period  is  relatively  long.  In  the  majority  of  the 
cases  the  eruption  appears  on  the  fourteenth  day  after  the  infection, 
sometimes  on  the  thirteenth  and  more  rarely  as  late  as  the  seventeenth 
or  even  the  nineteenth  day  and  in  some  cases  the  incubation  period  is 
given  as  four  weeks. 

As  a  rule  the  prodromes  are  unimportant  or  absent.  Thomas  and 
Henoch  say  that  in  most  cases  the  eruption  is  the  first  symptom  and, 
in  fact,  one  often  hears  from  the  most  anxious  and  observant  mothers 
that  nothing  was  noted  until  the  appearance  of  the  eruption.  Bohn, 
Gerhardt,  Cerf  and  many  French  authors  are  of  the  opinion  that  mild 
prodromes  are  the  rule.  Semtschenke  found  this  to  be  the  case  in  808 
cases  out  of  872  but  his  observations  were  made  in  a  Russian  orphan 
asylum  where  hygienic  conditions  were  not  of  the  best. 

The  prodromal  symptoms  last  only  one  or  two  days,  rarely  four  or 
five,  and  consist  of  fever,  anorexia,  restless  sleep,  general  malaise,  and 
sometimes  there  is  pain  in  the  abdomen,  vomiting  and  nose  bleed. 
Pain  in  the  joints  and  back  may  be  so  intense  as  to  suggest  variola. 
High  fever  is  noted  in  children  who  usually  have  high  temperature 
from  slight  causes  and  severe  nervous  symptoms  may  be  met  with  in 
some  cases.  Demme  has  noted  blood  in  the  stools  which  disappeared 
with  the  eruption.    . 


PLATE  18. 


a.     Eruption  of  varicella  (3  phases)  on  the  hand  and  forearm. 

h.     Glo^'e-Hke  desquamation  of  the  skin  of  the  hand  after  scarlet  fever. 

c.     Softening  of  gland  after  scarlet  fever. 


VARICELLA  335 

The  length  and  severity  of  the  prodromes  varies  and  it  must  be 
noted  that  a  patient  who  has  had  severe  prodromes  may  have  a  very 
favorable  and  short  course  of  the  disease. 

In  typical  cases  the  eruption  appears  on  the  scalp  and  face  and 
nearly  at  the  same  time  over  the  body.  There  arc  numerous  small 
round  spots  part  of  which  either  remain  small  or  disappear  altogether, 
the  remainder  enlarge  and  form  papules  about  the  size  of  a  pea.  A  small 
vesicle  forms  on  these  in  the  course  of  a  few  hours  and  this  may  increase 
greatly  in  size.  The  eruption  may  be  seen  in  all  stages  on  the  same 
patient  at  the  same  time.  The  picture  suggests  an  astronomical  map 
where  irregular  stars  of  various  sizes  are  situated  close  together 
(Heubner).  After  a  day  or  less  the  contents  of  the  vesicles  begins  to 
be  absorbed  and  in  a  couple  of  days  there  remains  onlj'  a  yellow- 
brown  or  black  scab.  This  drops  off  in  a  few  days  usually  without 
leaving  any  scar. 

As  a  rule  the  child's  general  condition  is  so  httle  disturbed  that  it 
is  with  difficulty  that  it  can  be  kept  in  bed.  There  is  usually  httle  or 
no  fever.  The  first  night  may  be  a  httle  restless,  the  appetite  poor  and 
after  that  the  child  feels  well  again. 

According  to  Thomas  and  Rille  there  is  nearly  always  some  tern^ 
perature  even  if  it  be  trifling  and  of  short  duration,  and  this  may  last 
two  or  three  days  or  even  much  longer.  The  author  has  observed  a 
case  where  there  was  continuous  fever  for  eleven  days.  There  is  no 
regular  temperature  curve  nor  does  the  severity  of  the  fever  depend 
on  the  amount  or  duration  of  the  eruption.  The  temperature  does  not 
furnish  any  differential  point  between  varicella  and  light  variola  cases. 
In  variola  there  is  a  fever-free  period  at  the  time  of  the  appearance  of 
the  eruption,  this  may  be  wanting  however  in  some  cases.  On  the  other 
hand  in  varicella  the  fever  may  chsappear  and  recur  later.  Fever  due 
to  suppuration  has  been  reported  by  Desandre  (1901)  Lanhartz  (1897) 
and  Comby. 

The  eruption  causes  but  trifling  inconvenience,  but  some  patients 
may  complain  a  gi-eat  deal  on  account  of  it,  especially  that  there  is 
sometliing  sticking  or  biting  them.  Itching  may  be  present  in  some 
cases. 

Severe  symptoms  may  come  on  late  as  well  as  in  the  prodromal 
stage,  even  death  may  result.  Fiirbringer  (1896)  has  reported  a  case 
of  undoubted  varicella  when  the  child  died  without  there  being  any 
apparent  comphcations. 

The  Exanthem. — There  is  no  great  difference  in  the  formation  of 
the  variola  and  the  variceUa  vesicle.  A  light  variola  may  resemble 
varicella  or  varicella  may  exceptionally  resemble  variola.  A  single 
vesicle  may  resemble  variola  in  an  otherwise  typical  varicella.  The 
varicella  vesicle  is  not  as  most  recent  descriptions  give  it  made  up  of 


33G 


THE   DISEASES   OF   CHILDREN 


a  single  chamber,  but  of  many  like  variola.  Primary  umbilication  is 
not  infrequently  seen  but  it  disappears  more  quickly  than  in  variola. 
Secondary  umbilication  occurs  from  the  drying  of  the  older  central 
part  more  quickly  than  the  newer  periphery. 

The  contents  of  the  vesicles  are  not  always  clear  throughout  but 
may  be  eitlier  watery,  milky,  purulent  or  even  haemorrhagic  and  second- 
ary suppuration  of  the  vesicle  is  not  infrequent.  The  hirmorrhagic 
and  purulent  forms  of  the  disease  will  be  considered  later.  More  rarely 
the  vesicle  becomes  filled  with  air,  which  is  drawn  in  through  the  injured 
epidermis  as  the  contents  of  the  vesicles  is  absorbed  (Windpocken, 
Varicella  ventosa,  siliquosa,  emphysematica). 

It  is  incorrect  to  state  that  there  is  no  stage  of  papules  and  inflam- 
matory infiltration  of  the  skin.  In  the  ordinary  course  of  tiie  disease 
the  physician  rarely  sees  the  papules  which  are  not  very  prominent 


Fig.  62. 


Section  through  a  twenty-four  hour  old  varicella  vesicle,  -nith  slightly  clouded  contents. 

and  of  short  duration.  Sometimes,  however,  papules  one  or  two  days 
old  may  be  noted.  Microscopic  sections  show  that  the  skin  is  always 
infiltrated  even  though  the  redness  is  scarcely  apparent  and  it  is  not 
uncommon  for  a  papule  to  attain  the  size  of  a  smallpox  papule  or 
vaccinia  pustule.  In  severe  cases  there  are  regions  of  the  body  on  which 
the  skin  between  the  pustules  is  swollen  and  of  an  erysipelatous  redness. 

The  absence  of  scarring  does  not  differentiate  varicella  and  variola. 
When  the  disease  is  protracted  or  when  there  is  secondary  infection, 
bad  treatment,  scratching  or  constitutional  disturbance,  the  healing 
may  be  delayed  and  there  may  be  destruction  of  the  skin  and  permanent 
scarring  may  result.  It  may  be  difficult  or  impossible  to  tell  the.se  scars 
from  smallpox  scars.  The  number  of  these  scars  is  seldom  great  and 
a  tendency  to  decrease  in  size  is  noted  as  time  goes  on. 

The   histologic   picture   varies.      If   one   chooses   typical   variceUa 


VARICELLA  337 

vesicles,  those  in  wliich  there  is  no  purulent  exudate  and  about  which 
there  is  no  infiltration,  and  compares  them  with  the  fully  developed 
smallpox  pustules,  the  difference  between  the  two  is  most  marked. 
If,  however,  one  chooses  the  varicella-hke  vesicles  from  a  Hglit  case  of 
smallpox  and  compares  them  to  a  typical  varicella  vesicle  or  on  the 
other  hand  compares  typical  variola  pustules  with  the  eruption  of 
varicella  variohformis,  one  finds  no  difference.  Unna  (1894)  at  least 
came  to  these  conclusions  as  a  result  of  his  investigations  and  lately 
Heubner  has  expressed  the  same  opinion.  By  examining  the  accom- 
panying figure  (Fig.  62)  kindly  lent  by  Professor  Riehl,  and  com- 
paring it  with  a  section  of  a  variola  vesicle,  one  sees  that  the  processes 
are  of  the  same  general  nature  and  differ  only  in  intensity  and  duration. 
The  vesicles  appear  usually  first  upon  the  scalp  and  face  but  often 
simultaneously  on  the  entire  body.     New  crops  of  vesicles  appear  from 

Fig.  63. 


Hemorrhagic  variola  in   a  ten-month-old  chikl  on   the   11th  day  of  the  eruption.    Note  the  eruption  in 

the  mouth.     (From  CorlettJ 

time  to  time  so  that  one  finds  all  stages  of  the  eruption  at  the  same  time 
on  the  same  part  of  the  body.  In  smallpox  the  eruption  appears  first 
on  the  face,  a  day  later  on  the  trunk  and  two  days  later  on  the  hands 
and  feet.  The  eruption  is  about  the  same  size  and  thickest  upon  the 
face  and  backs  of  the  hands  and  \\Tists.  In  irregular  cases  of  variola 
the  distribution  may  be  as  general  as  it  is  in  varicella  and  the  eruption 
may  appear  in  crops.  In  Fiiibringer's  case  (1896),  which  was  the  start- 
ing point  of  the  last  Berlin  epidemic,  the  diagnosis  of  chicken-pox 
was  made  upon  the  appearance  of  the  eruption  in  successive  crops,  on 
the  other  hand  varicella  is  seen  in  which  all  of  the  eruption  appears 
simultaneou.sly. 

The  eruption  may  all  come  out  in  one  day  but  as  a  rule  new  vesicles 
appear  the  next  daj'.  Sometimes  there  are  distinct  pauses  after  which 
new  crops  of  the  eruption  appear,  and  these  may  he  accompanied  by 
elevation  of  the  temperature.     Thomas  notes  a  case  where  new  vesicles 

11—22 


338 


THE   DISEASES   OF   CHILDREN 


Fig.   64. 


appeared  after  a   month's  time  Init  one  might  question  whether  this 
was  not  a  recurrence. 

It  is  well  known  that  eruptions  may  occur  in  variola  before 
the  appearance  of  the  regular  eruption  especially  when  there  is 
chemical  or  mechanical  irritation  of  some  part  of  the  body  as  from 
garters,  corsets  and  bandages.  Similarly  the  eruption  may  be  thickly 
set  in  varicella  especially  under  bandages,  from  the  irritation  of  dis- 
charges in   poorly-cared-for   cliildren,   and  in   the   geni to-crural   region 

from  the  irritation  of  the 
menstrual  flow.  The  pressure 
of  clotliing  sometimes  causes 
such  an  arrangement  of  the 
vesicles  as  to  lead  to  the  ap- 
pearance of  herpes  zoster, 
with  which  it  may  be  mis- 
taken. Bokay  (1892)  has  re- 
ported a  case  which  was 
treated  as  herpes  zoster  and 
later  in  the  same  family  there 
was  an  outbreak  of  varicella. 
Irritation  of  the  skin  may 
cause  varicella  to  run  a  much 
more  severe  course  than 
would  otherwise  be  the  case. 
Desoil  (1892)  had  a  case  in 
which  the  parents  of  the 
child,  acting  on  their  own 
authority,  gave  it  a  mustard 
bath  in  the  prodromal  stage. 
The  eruption  appeared  and 
there  were  from  500  to  600 
confluent  vesicles  which  sup- 
purated. These  healed  after 
about  six  weeks  with  very 
serious  scarring. 

The  number  of  varicella  vesicles  is  extremely  variable.  Thomas 
gives  10  as  a  mininmm  number  and  800  as  a  maximum.  Sometimes 
fewer  may  be  noted  and  a  single  one  may  be  all  that  can  be  found. 
These  cases  occur  in  children's  hospitals  where  there  is  a  house  epidenric 
and  where  very  careful  examination  of  the  children  is  undertaken. 

Confluent  eruptions  may  sometimes  be  observed.  In  almost  every 
case  the  confluence  of  a  few  vesicles  may  be  noted.  There  have  been 
numerous  reports  of  cases  in  which  the  eruption  was  confluent  in  the 
same  way  as  in  smallpox. 


Scars  around  waist  in  a  seven-year-old  boy,  six  years 
after  an  attack  of  varicella.  The  pressure  of  the  clothing 
produced  a  great  number  of  large  pustules,  while  the  erup- 
tion was  slight  on  other  parts  of  the  body. 


VARICELLA 


339 


Fig.  6.5. 


The  size  of  the  vesicles  also  varies  greatly.  In  some  cases  they 
are  the  size  of  a  pin  head  and  Thoma.s  and  Henoch  speak  of  "miliary" 
vesicles.  The  average  size  is  that  of  a  lentil,  and  vesicles  with  an  average 
diameter  of  10  mm.  are  not  uncommon.  They  may  be  the  size  of  a  dollar. 
(Thomas,  Demme)  and  a  case  was  described  in  wliich  vesicles  10  cm. 
in  diameter  occurred  (Geddings,  1885).  In  such  cases  one  has  the 
pemphigus-like  or  bullous  form  of  varicella  which  is  to  be  differentiated 
from  the  accidental  occurrence  of  varicella  and  pemphigus  at  the  same 
time. 

Abortive  and  ruchmentary  forms  of  the  eruption  may  be  noted  in 
wliich  the  exanthem  comes  to  a  standstill  before  vesicles  are  formed. 
There  may  be  a  simple  roseola  wliich 
disappears.  Thomas  described  this 
form  as  Roseola-  Varicellosa\  It  may 
happen  that  the  eruption  goes  as  far  as 
papule  formation  and  then  disappears. 
Gaillard  reported  an  interesting  case 
in  which  there  were  papules  in  the 
skin  and  numerous  vesicles  upon  the 
mucous  membranes.  These  vesicles 
occasioned  great  pain. 

The  eruption  even  in  mild  cases 
occurs  on  the  mucous  membranes  but 
not  so  regularly  as  in  variola.  Comby 
has  made  especial  studies  of  the  enan- 
them  and  finds  that  it  usually  begins 
before  the  exanthem  but  sometimes  at 
the  same  time  or  after  it. 

Location. — It  is  noted  frequently  in 
the  mouth,  on  the  hard  palate,  the 
tongue,  gums,  and  also  on  the  tonsils 
and  pharynx.  The  number  of  vesicles  is  usually  small  and  one  may  find 
but  a  single  spot  but  sometimes  the  vesicles  are  more  numerous  than 
those  of  the  exanthem.  One  does  not  often  see  the  vesicular  stage  of 
the  enanthem  as  the  eruption  seems  to  develop  more  quickly  in  the 
mouth  than  on  the  skin.  Usually  at  the  time  of  the  first  examination 
of  the  mouth  the  covering  of  the  vesicle  has  been  partly  or  entirely 
destroyed  by  the  warmth  and  action  of  the  mouth  secretions  and  one 
sees  instead  of  a  vesicle  an  erosion  varying  in  size  from  that  of  a  millet 
seed  to  a  pea,  yellowish  white  in  color  anil  surrounded  by  a  red  zone. 
These  can  be  differentiated  from  ordinary  aphtha^,  when  remains  of 
the  top  of  vesicles  are  still  present.  These  seem  to  give  rise  to  but 
little  discomfort  in  most  children  but  some  complain  on  chewing  and 
swallowing.     When  the  vesicles  are  numerous  and  inflamed,  and  there 


\ 


/ 


Varicella  enanthem  in  t!ie  moutli  of  a  five-year- 
old  girl.    Third  day  of  eruption. 


340 


THE   DISEASES   OF   CHILDREN 


Flc.  00. 


is  a  secondary  stomatitis,  there  may  be  great  pain  on  swallowing,  a 
severe  burning  sensation  in  the  throat  anil  other  similar  sensations. 
Generally  the  simple  lesions  heal  rapidly  and  the  severer  manifesta- 
tions may  be  favorably  influenced  by  suitable  treatment. 

The  suppurative  infiltration  of  the  ulcerations  may  be  the  cause 
of  a  severe  tonsillitis  with  fever.  Girode  (1893)  has  described  a  case 
of  pseudomembranous  angina  due  to  the  streptococcus  occurring  in 
the  course  of  varicella.  The  fever  lasted  eight  days  with  severe  general 
symptoms  and  there  was  a  complicating  orchitis.  Perforation  of  the 
soft  palate  from  an  ulcerating  varicella  pustule  has  also  been  observed 
(Kaupe,  1903). 

Involvement  of  the  eye  is  not  infrequent  and  this  generally  consists 
of  a  vesicle  on  the  edge  of  the  lid  or  upon  either  the  ocular  or  palpebral 

conjunctiva.  This  gives  rise  to  great 
.discomfort  and  suffering  on  the  part 
of  the  patient  and  may  result  in  a 
phlegmon  of  the  lid.  More  rarely  the 
cornea  may  be  involved.  This  comes 
on  with  marked  inflammation  and  in 
favorable  cases  healing  takes  place  with 
a  clouding  of  the  cornea.  In  unfavor- 
able cases,  the  inflammation  extends 
into  the  eye  as  it  so  frequently  does 
in  smallpox.  [For  literature  see 
Oppenheim  (1905)  and  Cerf  (1901)]. 
It  is  not  unusual  for  the  varicella 
vesicles  to  form  in  the  auditory  canal. 
I  once  saw  vesicles  in  opposite  ends 
of  the  canal  causing  occlusion  accom- 
panied by  great  pain,  deafness,  and 
tinnitus.  Attention  may  be  drawn  to 
the  presence  of  vesicles  in  the  nose  by  nasal  haemorrhage.  Sometimes 
a  purulent  inflammation  follows  and  for  weeks  there  may  be  bloody 
and  purulent  discharges  which  form  crusts  in  the  nose  and  these 
greatly  interfere  with  breathing. 

The  eruption  is  more  frequent  on  the  genitalia  of  girls  than  of  boys. 
In  the  former  it  is  located  on  the  labia  while  in  the  latter  it  is  seen  on 
the  glans  or  prepuce.  In  boys  discomfort  is  rare  (Coombs  described 
a  16  hour  anuria)  but  in  girls,  vulvitis,  painful  urination  or  even  anuria 
may  be  observed.  Through  scratching  or  uncleanliness,  ulcers,  phleg- 
mons, necrosis,  lymphadenitis  and  even  general  infection  may  result. 
Of  especial  importance  is  the  occurrence  of  the  eruption  in  the 
larynx  and  trachea.  This  has  been  fully  described  by  French  authori- 
ties, notably   by  Harlez   (1898)   Marfan  and   Halle   (1896),   Roger  and 


Varicella  enaiulii-iii  .il>->iii    t  m.-  su«*;i  i 
same  child.    Third  day  of  eruption 


VARICELLA  341 

Bayeux  (1898)  and  Lannoise  (1896).  The  symptoms  are  like  those  of 
a  severe  case  of  croup,  hoarseness,  a  barking  cough,  dyspnoea,  cyanosis, 
smothering  attacks  and  asphyxia.  Intubation  and  tracheotomy  may 
be  necessitated  but  sometimes  the  patient  is  beyond  helping.  Cerf 
collected  seven  cases,  four  of  which  died.  The  diagnosis  in  the  early 
stages  may  be  impossible  owing  to  the  difficulties  of  laryngoscopic 
examinations  in  young  children. 

This  may  be  tlie  case  where  the  trouble  in  the  larynx  begins  before 
the  appearance  of  the  eruption  as  frequently  happens.  Without 
an  inspection  of  the  larynx  one  can  never  be  sure  there  is  not  a 
compHcating  diphtheria  and  the  early  use  of  diphtheria  antitoxin 
is  advisable. 

Prodromal  rashes  are  rare  in  varicella  but  in  some  epidemics  they 
may  be  quite  frequent.  Henoch  has  described  prodromal  rashes  re- 
sembhng  scarlet  fever  coming  on  several  hours  before  varicella  rash. 
Thomas  noted  a  similar  rash  fifteen  hours  before.  Fleischmann  (1870) 
observed  a  measles-like  prodromal  rash  lasting  forty  eight  hours.  Cerf 
has  collected  forty  five  cases  of  prodromal  varicella  rashes. 

As  a  rule  these  rashes  appear  from  two  to  twenty-four  hours  before 
the  vesicles  but  rashes  simultaneous  with  or  appearing  after  the  vesicles 
have  been  reported.  At  the  same  time  as  the  appearance  of  these  rashes, 
or  some  hours  before,  there  are  often  high  fever,  vomiting,  diarrhoea, 
loss  of  appetite,  headache,  dizziness,  joint  pains,  and  difficulty  of  swal- 
lowing. Burning  sensations,  itching  and  subsecjuent  desciuamation  are 
not  observed.  About  six-sevenths  of  the  prodromal  rashes  in  varicella 
resemble  scarlet  fever,  the  others  are  like  measles,  ha^morrhagic  or 
mixefl.  The  rash  rarely  covers  the  entire  body  and  areas  of  normal 
skin  may  usually  be  noted.  The  color  of  the  rash  is  generally  a  uniform 
bright  red,  more  rarely  either  pale  or  livid  red.  These  rashes  last  on  an 
average  about  twenty-four  hours,  often  less,  but  they  may  remain  for 
two  days  or,  in  exceptional  cases,  for  five  or  six  clays.  In  many  cases 
where  there  are  prodromal  rashes  there  are  severe  general  symptoms 
or  complications. 

But  few  authors  ascribe  any  specific  odor  to  A^aricella.  Heim,  how- 
ever, was  of  the  opinion  that  it  had  a  distinctive  odor  quite  different 
from  that  of  variola. 

Complications  and  Sequelae.  — The  complications  and  sequela*  of 
varicella  are  rare  but  nevertheless  are  as  numerous  in  variety  as  those 
met  with  after  other  infectious  diseases.  Nephritis  is  the  most  impor- 
tant of  the  complications.  This  was  known  from  very  early  times  but 
the  first  important  observations  were  made  by  Henoch  in  1884.  The 
nephritis  following  varicella  is  rarer  and  more  benign  than  that  follow- 
ing most  of  the  acute  infectious  diseases.  There  may  be  little  to  call 
attention  to  the  condition  and  it  may  disappear  without  being  detected 


342  THE    DISEASES   OF   CHILDREN 

unless  urinary  examinations  are  made  as  a  matter  of  routine.  The 
cases  may  be  divided  into  three  classes  according  to  their  intensity. 
Ungcr  and  later  Cerf  have  made  the  following  divisions:  (1)  latent 
nephritis  in  which  there  are  no  symptoms  and  albuminuria  is  only 
discovered  when  looked  for;  (2)  light  nephritis  in  which  there  is  marked 
albuminuria  and  some  cedema  but  no  severe  symptoms  and  (3)  severe 
nephritis  with  fever,  marked  albuminuria,  anuria,  cramps,  gastro- 
intestinal disturbances,  uraemia,  etc. 

In  certain  epidemics  nephritis  is  especially  frequent.  It  is  noted 
usually  after  the  vesicles  are  dried  up  and  it  is  important  to  note  that 
a  very  severe  nephritis  may  follow  a  light  .attack  of  varicella. 

Precautions  against  nephritis,  such  as  long  rest  in  bed  and  a  milk 
diet,  owing  to  the  rarity  of  the  complication  are  rarely  employed.  Chil- 
dren who  have  previously  had  nephritis  should  have  all  such  precautions 
taken.  In  all  cases  where  there  is  nephritis  the  treatment  should  be 
undertaken  in  earnest  as  a  severe  nephritis  may  otherwise  result. 

Arthritis  varicellosa  may  occur  during  the  eruptive  period  or  later. 
It  is  usually  polyarticular  but  only  one  joint  may  be  affected.  It  may 
start  acutely  or  it  may  come  on  gradually.  There  are  two  forms,  a  simple 
serous  form  and  a  severe  suppurative  form.  This  last  may  follow 
secondary  infections  by  pus  germs,  or  occur  through  general  blood  in- 
fection or  through  the  lymphatics  from  some  neighboring  site  of  infec- 
tion. The  prognosis  in  every  case  must  be  guarded  owing  to  the  danger 
of  general  infection. 

Complications  involving  the  nervous  system  are  much  less  frequent 
after  varicella  than  after  the  other  infectious  diseases.  W.  Gay  (1894) 
observed  a  case  of  paraplegia  with  loss  of  power,  sensibility  and  reflexes 
of  the  legs.  This  occurred  in  a  boy,  two  and  one-half  years  old,  fourteen 
days  after  a  normal  varicella.  Recovery  took  place  in  three  weeks. 
Under  similar  circumstances  Marfan  noted  a  case  of  monoplegia  which 
affected  the  arm  and  also  a  case  of  external  ophthalmoplegia  of 
muscular  origin.  Chorea,  multiple  sclerosis  and  encephalitis  have  also 
been  reported. 

Secondary  infections  with  pus-forming  bacteria  are  important. 
It  is  not  infrequent  for  most  of  the  vesicles  to  be  infected  and  become 
pustules.  This  may  occur  in  well-cared-for  children  l)ut  more  often 
happens  in  the  weak  and  poor.  Scratching  and  uncleanliness  are  the 
most  common  causes  but  crust  pustules  are  the  rule  in  the  regions  soiled 
by  the  urine  and  stools  in  uncleanly  children.  Irritating  applications 
may  also  cause  pustules.  The  pustules  run  a  longer  course  than  the 
vesicles,  reaching  maturity  in  from  (i  to  10  ilays.  They  are  surrounded 
by  a  red  inflamed  area  and  in  the  middle  there  is  a  reddish  brown  umbili- 
cation  so  that  it  resembles  a  variola  pustule.  These  are  designated  by 
the  French  as  "la  pustule  en  cocarde."    Three  weeks  or  even  a  month 


VARICELLA 


343 


may  elapse  before  the  crust  falls  off.  The  general  symptoms  may  be 
severe  and  these  cases  may  be  mistaken  for  smallpox.  Local  infiani- 
mations  as  phlegmons,  furuncles,  sul)cutaneous  abscesses  and  erysipelas 


Fig.   67 


Variola-like  eruption  in  varicella  in  a  twu  and  a  lialf-ycar-uld  ijuj  .    Tlii.?  ca:^e  left  over  300  ^car^. 

may  occur  in  the  course  of  the  disease.  General  infection  may  result, 
with  other  local  manifestations,  in  thrombosis  of  the  vessels  and  also 
severe  general  symptoms.  Amongst  other  things  the  following  have 
been  reported:  osteomyelitis,  gangrene  of  both  legs  after  obliteration 
of  the  arteries,  suppurative  phlebitis  of  the  saphena,  suppurative  peri- 


344 


THE    DISEASES    OF   CHILDREN 


carditis,  otitis  media,  meningitis,  brain  and  lung  abscesses,  empyema, 
and  thyreoiditis. 

Gangrene  of  tlie  skin  may  be  observed  in  the  course  of  any  of  the 
acute  exanthematous  diseases  but  in  none  so  frecjuently  as  varicella. 
There  is  much  concerning  the  process  which  is  obscure  notwithstanding 
the  fact  that  the  gangrenous  form  has  been  known  since  1807  (Whitley 
Stokes),  according  to  Hesse  since  1691  (Gideon  Harvey),  and  has  been 
thoroughly  studied  by  many  observers.  Doubtless  underfed,  cachectic 
and  tuberculous  children  and  especially  those  weakened  by  diseases 
(measles,  whooping-cough,  pneumonia  and  the  Uke)  are  prone  to  gan- 
grene, but  sound  healthy  children  may  be  affected  as  well. 

The  bacteriological  investigations  have  not  thrown  much  hght  upon 
the  subject.  Staphylococci  and  streptococci  are  the  usual  find;  but 
virulent  diphtheria  bacilh  have  been  noted  in  a  case  (A.  Krjukoff,  1899)) 
and  in  Demme's  case  (1892)  the  ulcerations  were  of  a  tuberculous  char- 
acter. 

Fig.  68. 


Gangrenous  varicella  in  a  two  and  a  lialf-year-old  child.    Death  on  the  twelfth  day  o!  the  disease. 


Gangrene  may  be  noted  in  cases  running  an  apparently  normal 
course,  as  well  as  in  those  which  from  the  beginning  show  either  unusual 
or  very  severe  symptoms.  This  complication  generally  occurs  sporadi- 
cally in  an  otherwise  benign  epidemic  but  sometimes  there  are  numer- 
ous cases.  Heim  (1809)  leported  that  in  some  epidemics  there  was  an 
especial  tendency  to  gangrene.  The  gangrene  may  begin  on  the  first 
day  (Edwards,  1903)  or  even  as  late  as  the  drying  up  stage.  It  may 
affect  only  one  spot  or  the  majority  of  the  papules. 

Excluding  numerous  variations  the  course  is  about  as  follows: 
Whilst  the  remainder  of  the  eruption  continues  its  accustomed  course 
some  of  the  spots  suddenly  become  surrounded  by  a  large  inflamed 
area  and  the  contents  of  the  spots  become  hemorrhagic.  Later  the 
black  crusts,  which  have  resulted  from  the  drying  of  the  contents  of 
the  vesicles  fall  off  leaving  sharp-edged  ulcers  having  a  punched  out 
appearance.  The  bottom  of  the  ulcer  is  covered  with  discolored  pus 
or  small  cheesy  masses.    The  size  of  the  ulcerations  varies.    They  may 


VARICELLA  345 

remain  about  the  size  of  a  pea,  or  may  enlarge  to  the  size  of  a  dollar  or 
even  become  as  large  as  a  saucer.  Enormous  areas  of  ulceration  may 
result  from  the  union  of  several  ulcers  and  the  deeper  tissues,  fascia 
and  muscles  may  be  involved.  Spivak  (1895)  has  described  destruction 
of  the  scrotum  following  varicella. 

Varicella  may  predispose  to  tuberculosis  in  the  same  way  that 
measles  does.  One  may  see  a  child  with  a  latent  tuberculosis  which  may 
be  started  into  an  active  process  by  an  attack  of  varicella. 

Diagnosis. — Tliis  is  as  a  rule  easy,  as  variola  is  the  only  disease 
which  causes  any  real  difficulty.  Well-marked  smallpox  cases  offer 
no  difficulty,  even  to  those  who  know  the  disease  from  books  only,  nor 
does  typical  varicella  cause  any  trouble,  but  at3'pical  varicella  and  the 
lighter  irregular  forms  of  variola,  such  as  occur  after  vaccination  and 
revaccination,  may  be  impossible  to  differentiate.  The  differences  have 
already  been  fully  considered.  Of  greatest  importance  are  the  absence 
of  prodromes  and  the  occurrence  of  all  stages  of  the  eruption  at  the 
same  time.  In  doubtful  cases  there  are  only  two  criteria  which  may 
be  relied  upon:  The  origin  of  the  case  from  variola  and  the  capabiUty 
of  its  causing  the  disease  in  others.  A  doubtful  case  of  varicella  should 
only  be  regarded  as  such  in  the  absence  of  any  possibiUty  of  smallpox 
infection.  The  methods  of  diagnosis  on  an  etiological  basis  are  unfortu- 
nately not  of  value  to  the  practicing  physician. 

The  differential  diagnosis  from  other  diseased  conditions  must  be 
made  but  tliis  usually  presents  no  difficulties.  All  diseases  which  may  be 
confused  with  varicella  or  variola  must  be  considered:  Herpes,  eczema, 
pemphigus,  impetigo,  scabies,  varicella,  syphilitica,  urticaria  vesiculosa, 
drug  eruptions,  erythema  exudativum  multiforme  and,  in  the  beginning, 
rubeola,  measles  and  scarlet  fever.  In  all  cases  the  resemblance  is  a 
passing  one  and  careful  investigation  will  prevent  any  mistake. 

An  exception  must  be  made  in  unusual  cases  of  pempliigus.  Vari- 
cella especially  in  the  newborn  may  cause  a  well-marked  pempliigus, 
and  it  may  be  difficult  or  impossible  to  tell  this  from  other  forms  of 
pemphigus.  The  longer  duration  of  the  true  pempliigus  eruption  and 
the  occurrence  of  varicella  in  other  members  of  the  family  are  points 
of  importance.  Sometimes  in  the  papulopustular  stage  of  erythema 
exudativum  multiforme  the  diagnosis  cannot  be  made  for  a  day  or  two 
(see  Fig.  69). 

Prognosis. — Varicella  is  usually  regarded  as  such  a  harmless  dis- 
ease that  little  attention  is  paid  to  it  either  by  the  laity  or  physicians- 
In  spite  of  the  fact  that  almost  all  cases,  even  those  with  severe  symp- 
toms, end  favorably,  there  are  still  a  sufficient  number  of  unfavorable 
cases  which  should  deter  one  from  giving  an  unqualifietlly  gooti  prog- 
nosis. Experience  teaches  us  that  weak  newborn  infants,  and  those 
suffering  from   bowel   or  lung   diseases   and   above   all   tuberculous   or 


346 


THE   DISEASES   OF   CHILDREN 


Fig.  60. 


scrofulous  children  may  be  made  dangerously  ill  by  varicella,  especially 
when  the  patient  is  living  in  unhygienic  surroundings.  Fatalities  are 
rare  but  may  now  and  then  result  fropi  the  severity  of  the  infection, 
from  the  location  of  the  eruption,  as  in  varicella-croup,  and  from 
secondary  infections  or  complications. 

Treatment. — In  most  cases  treatment  is  unnecessary.  As  long  as 
there  is  fever  or  new  papules  appear  the  patient  should  be  kept  in  bed. 
As  soon  as  the  eruption  has  dried  and  an  examination  of  the  urine 
shows  that  the  kidneys  are  not  involved  the  child  may  leave  the  room. 

The  fever  is  rarely  so  high 
as  to  necessitate  any  drugs 
directed  to  the  reduction  of 
the  temperature.  Cooling 
draughts,  cold  compresses  to 
the  head  and  sponging  with 
cool  water  is  all  that  is  neces- 
sary. If  severe  brain  symp- 
toms come  on,  lukewarm  baths 
up  to  five  minutes  in  length, 
followed  by  spraying  with 
colder  water,  may  be  used. 

The  formation  of  ulcers  in 
the  throat  may  render  some 
treatment  necessary  as  the 
pain  often  interferes  with  the 
proper  nourishment  of  the 
child.  Rinsing  the  mouth  with 
marshmallow  tea  to  which 
borax  or  boric  acid  has  been 
added  may  be  employed. 

All  solutions  containing 
alcohol,    ethereal    oils    or    irri- 


tating  substances   as    well    as 


.Li.iihij  f.u-t  iil.v- jiL  a  Mil- \rar-old  girl  after 
eatiiiR  decayed  fruit. 


mechanical  cleansing  of  the 
mouth  with  a  tooth  brush 
should  be  forbidden.  In  exceptional  cases  the  pain  is  so  great  as  to 
necessitate  the  painting  of  the  ulcer  with  a  2-4  per  cent,  solution  of 
cocaine  (followed  by  rinsing  of  the  mouth). 

Great  cleanliness  should  be  exercised  about  the  genitalia  and  if 
there  is  any  inflammation,  ointments  containing  boric  acid  or  thymol 
should  be  used. 

Daily  baths  may  be  given,  but  all  rubbing  and  irritation  of  the  skin 
should  be  carefully  avoided  and  too  hot  water  should  not  be  used. 

Itching,  if   troublesome,  may  be  allayed   by  lukewarm  baths,  fol- 


PLATE  19. 


VARICELLA  347 

lowed  by  dusting  with  some  bland  powder  or  by  sponging  with  water 
to  which  vinegar  or  alcohol  has  been  added.  Internal  medications 
may  be  necessary  in  some  cases,  antipyrin,  phenacetin  or  even  morphia. 

The  hands  should  bo  kept  clean  and  the  finger  nails  should  receive 
special  attention.  If  scratching  is  "not  controlled  by  tlie  child  itself 
the  hands  or  liands  and  arms  siiould  be  restrained. 

Infections  of  the  skin  and  abscesses  should  be  treated  according 
to  ordinary  surgical  principles. 

The  child  should  be  watched  during  convalescence  antl  if  it  does 
not  recover  its  strength,  careful  regulation  of  the  diet,  tonics  and  even 
a  change  of  chmate  may  be  advised. 

Finally,  in  all  cases  in  which  variola  is  suspected,  and  in  varicella, 
occurring  in  adults,  vaccination  should  be  practiced. 


VACCINATION 

BY 

Bu.  L.  VOIGT,  OF  Hambukq 

TR.iNSLATED    BY 

Dr.  JOHN   RITHRAH,  Baltimore,  Md. 


By  vaccination  (from  vacca,  cow)  is  meant  the  conveying  of  the 
contents  of  the  pustules  of  cow-pox  found  on  the  udder  of  the  cow  to 
man  in  order  to  protect  him  from  variola.  Cow-pox  is  not  a  separate 
tlisease  but  originates  by  the  infection  of  the  cow  with  the  virus  of  the 
smallpox  of  man  or  of  animal  pox,  as  the  horse-pox.  When  the  virus 
is  transferred  to  the  cow  for  the  first  time,  smallpox  may  be  reproduced 
in  man  from  the  contents  of  the  pustules  but  after  two  or  three  trans- 
fers the  virus  becomes  attenuated  until  it  is  no  longer  possible  to  get 
smallpox  from  it  by  inoculating  man,  and  this  is  named  variola  vaccine. 

Before  Jenner's  time  innoculation  of  cow-pox  had  been  resorted 
to  here  and  there  for  the  protection  of  man  from  smallpox,  but  to  him 
belongs  the  honor  of  first  having  made  the  matter  clear  and  of  having 
given  it  widespread  publicity.  Jenner's  attention  was  drawn  to  the 
question  whilst  he  was  yet  a  student  by  the  remark  of  a  young  woman 
"I  cannot  get  smallpox  because  I  have  had  cow-pox."  After  years 
of  study  Jenner  made  a  decided  step  when  on  May  14,  1796,  he  practiced 
vaccination  on  a  boy  named  Phipps  with  the  virus  from  a  pustule  on 
the  hand  of  a  milk-maitl  Sarah  Nelmes,  who  had  stuck  her  hand  with 
a  thorn  and  subsequently  had  it  infected  with  cow-pox  whilst  milking. 
With  this  virus  Jenner  vaccinated  a  number  of  children  including  his 
own  son,  and  then  inoculated  them  with  smallpox  virus  and  none  of 
the  children  developed  smallpox.  After  a  year  antl  a  half  Jenner  pub- 
lished his  results  in  a  brochure  "An  incjuiry  into  the  causes  and  effects 
of  the  variohe  vaccinte"  London  1798.  In  this  paper  Jenner  described 
chicken-pox  and  its  relation  to  other  animal  poxes  especially  horse- 
pox,  and  the  effect  of  vaccinating  man  and  the  immunity  so  produced. 
Jenner  stated  that  persons  who  have  had  cow-pox  are  protected  from 
smallpox,  either  natural  infections  or  inoculation,  just  as  those  are 
who  have  had  smallpox  itself. 

With  the  spread  of  vaccination,  smallpox  became  less  frequent 
and  the  dangerous  practice  of  inoculation  fell  into  disuse.  There  were 
those  who  hoped  to  wipe  out  variola  entirely.  As  early  as  1807,  Bavaria 
made  it  a  law  that  all  newborn  children  should  be  vaccinated  and  in 

348 


VACCINATION  349 

1816  Sweden  passed  similar  laws.    Subscciuently  in  most  of  the  German 
states  not  only  vaccination  but  rcvaccination  was  ordered. 

Smallpox  became  a  rare  disease,  at  least  in  Germany,  and  in  the 
second  decade  of  the  last  century  there  were  as  there  are  to-day  many 
young  physicians  who  had  never  seen  the  disease.  About  the  twenties 
when  the  protection  afforded  by  vaccination  began  to  wear  off  there 
began  to  be  mild  cases.  Four  decades  passed  with  here  and  there  mild 
epidemics  of  smallpox  until  1860-1870  when  a  pandemic  of  consider- 
able severity  occurred  in  Germany.  The  enormous  loss  of  life  during 
this  epidemic  led  to  the  passage  of   the  law  of    1874  (Germany)  which 

Fig.  70. 


The  cytoryctes 
in  the  cell 
protoplasm. 


Vaccinal  changes  in  the  corneal  cells  of  a  rabbit. 

provided  for  vaccination  during  the  first  5'ear  of  life  and  during  the  twelfth 
year.  Germany  has  since  then  remained  free  from  smallpox.  Other 
countries  now  having  laws  modelled  after  the  German  law  are  Hungary, 
Italy,  Japan,  Portugal,  Spain,  France,  Turkey,  Argentine  Repubhc, 
Brazil  and  Mexico.  In  Japan  and  Turkey  every  inhabitant  must  be 
revaccinated  every  five  years.  In  France  a  second  vaccination  is  pro- 
vided for  in  men  and  women  and  in  Germany  the  men  are  revaccinated 
on  entering  the  army.  In  Austria  vaccination  is  practiced  on  entering 
school  and  the  army.  The  old  laws  of  Denmark  antl  Scandinavia  have 
been  retained.  Russia  is  behind  the  times  and  the  laws  of  Switzerland. 
Belgium  and  Hollantl  do  not  afTord  adequate  immunity.  England,  the 
birth-place  of  vaccination  had  in  1857  a  law  providing  for  the  vaccina- 
tion of  infants  during  the  first  four  years  of  life  but  for  no  subse(iuent 


350 


THE   DISEASES  OF   CHILDREN 


vaccination.  Tliis  law  lias  become  voitl  and  in  1898  a  so-called 
"conscience  clause"  was  added  permitting  those  who  did  not  want  to 
have  their  children  vaccinated  to  refuse.  Smallpox  naturally  prevails 
in  England.  The  bovine  vaccination  which  is  without  danger  is  pre- 
ferred to  the  human  inoculation  which  was  formerly  em])loyed  and  now 
forbidden  because  danger  to  life  in  the  latter  is  entirely  excluded.  Then 
too  its  contagion  was  carried  by  the  air  while  that  of  the  cow-pox  was 
only  possible  by  direct  transmission  to  the  denuded  skin. 

AVhen  the  infectious  material  of  cow-pox  or  smallpox  reaches 
the  deeper  layers  of  the  epithelium  certain  characteristic  changes  take 
place  chiefly  in  the  cell  protoplasm  and  later  there  is  cell  necrosis.    The 

FiQ.  73. 


Flu.   71. 


Strongly   developed    vaccmation   pustule,   seven 
days  after  vaccination. 


Revaccination  pustule,  seven  day.s  after  vac- 
cination. 


beginning  of  these  changes  is  best  studied  in  the  cornea  of  the  rabbit 
where  there  are  no  blood  vessels.  The  characteristic  appearance  in 
these  cells  permits  a  differential  diagnosis  between  smallpox  and  cow-pox. 
These  changes  in  the  cells  were  thought  by  some  to  be  protozoa  and  the 
term  cytoryctes  was  applied  to  them  by  Guarnieri.  Others  consider 
them  due  to  the  causative  agent  or  toxin  of  smallpox  or  cow-pox. 

Since  the  time  of  Jenner  in  vaccinating  man  the  inoculation  has 
been  made  on  the  arm  in  the  region  of  the  insertion  of  the  deltoid.  The 
development  of  the  le.sion  is  similar  to  that  of  variola,  that  is  a  papule, 
then  a  vesicle,  and  pustule,  but  the  eruption  is  localized  after  an  inocu- 
lation of  cow-pox  and  not  generalized  as  in  smallpox.  There  is  reticular 
and  balloon  degeneration  of  the  epithehum  and  an  influx  of  leucocytes. 
The  vesicle  is  higher  at  its  border  than  it  is  in  the  centre  and  as  in  the 
variola  pustule  it  is  umbihcated.    The  walls  and  upper  part  of  the  floor 


VACCINATION 


351 


of  the  pustule  become  necrotic  and  the  fluid  part  of  the  pustule  dries 
and  forms  a  crust  or  scab.  Under  this  scab  is  found  a  scar,  which 
appears  covered  with  dehcate  reddish  skin  when  the  scab  drops  off  but 
which  later  becomes  hghter  in  color  and  remains  plainly  visible.  On 
the  seventh  day  after  the  inoculation  the  vaccine  pustule  has  a  silver 
gray  color,  is  on  a  sUghtly  reddened  base  or  one  not  reddened  at  all, 
and  contains  clear  Ij'mph,  which  as  a  rule  is  sterile.  The  following  day 
the  h'mph  is  cloudy  and  contains  skin  bacteria.  Up  to  the  ninth  day 
the  inflammation  increases  and  the  axillary  l3-mph-nodes  swell,  but  on 
the  eleventh  day  the  process  begins  to  diminish  and  the  scab  falls  off 
between  the  twenty-first  and  twenty-eighth  day,  thus  ending  the  pro- 
cess.    Between  the  sixth  and  ninth  day  there  is  usually  a  fever,  the 


Fig.  73. 

!■ 

pp 

^.-i<^>" 

^^5 

i^^^'^'f^nH 

^ 

r 

^-".■^^"^^Hl 

■ 

r 

^ 

f0mm  ^ 

F 

v*^- .,_  -^     '^^^^^^1 

L 

^^k 

I- 

m 

Wt 

- 

1 

^m 

1 

I 

r 

m 

Secondary  vaccinia.    Eight  days  after  vaccination. 


temperature  rises  to  39.5°  C.  (103°  F.),  sometimes  to  40°  C.  (104°  F.), 
and  the  individual  feels  ill.  In  some  instances,  a  trace  of  albumin  may 
be  detected  in  the  urine.  After  a  successful  vaccination,  revaccination 
will  not  "take"  but  after  five  years  have  elapsed  from  the  time  of  the 
vaccination  or  an  attack  of  varioloid  the  susceptibihty  to  the  vaccine 
virus  begins  to  return  and  grows  stronger  and  stronger  as  time  goes  on. 
The  vaccination  at  the  twelfth  year,  practiced  in  Germany,  often  fails 
to  take,  or  there  is  a  trifling  redness  of  the  epidermis  wliich  disappears 
on  the  seventh  day.  In  most  cases  there  is  the  formation  of  a  papule 
and  pustule  but  the  course  is  more  rapid  and  corresponds  to  the  small- 
pox as  modified  by  vaccination,  the  so-called  varioloid. 


352  THE    DISEASES   OF    CHILDREN 

In  those  vaccinated  for  the  first  time  there  may  be  some  congestion 
of  the  skin  but  no  real  eruption.  During  tlie  summer  months  and  in 
those  who  have  delicate  skins  eruptions  may  occur.  Sometimes  the 
rash  resembles  measles  or  German  measles,  sometimes  it  is  an  urticaria, 
rarely  a  general  vaccinia  eruption  may  occur  consisting  of  small  vaccine 
vesicles.  All  of  these  disappear  wlicn  the  period  of  immunity  is  reached, 
that  is,  at  the  end  of  the  second  week. 

Small  vesicles  may  develop  near  the  original  vesicle  evidently  by 
extension  through  the  lymph  channels  and  secondary  vaccination  may 
occur  through  wounding  of  the  vesicle  and  accidental  infection  on  other 
parts  of  the  body  or  on  other  persons.  These  secondary  vaccinations 
may  cause  httle  or  great  suffering  according  to  their  location  (see  Fig. 
73).  Panophthalmitis  may  be  caused  by  the  vaccine  virus  getting 
into  the  eye  of  an  unvaccinated  person.  If  the  virus  gets  into  an  exposed 
surface,  as  a  weeping  eczematous  area,  a  dangerous  illness  may  ensue. 
The  physician  should  take  care  to  prevent  tliis  and  warn  the  parents 
Fig.  74.  of  cluldreu  Suffering  witli 


impetigo,  moist  eczema, 

urticaria,     furunculosis, 

^^^^^^^^^^s.       ^s  well   as  of  scrofulous 

and  weak  children,  that 
in  these  children  vacci- 
nation is  not  free  from 
danger. 

The  treatment  of  tlie 

1. — Lancet.      2. — Weichardr.s   vaceiiiatiuti    needle.     3. — Tlie  same  SVUlPtOmS       wllicll       ma^' 

in  case.    4. — Lindenbom's  platino-iridium  lancet.  ^ 

appear  after  vaccination 
requires  a  word.  If  the  fever  is  high  the  patient  should  be  sponged  with 
lukewarm  water  or  even  bathing  may  be  resorted  to.  The  vesicle  must 
not  be  injm-ed  and  should  be  allowed  to  dry  without  opening.  It  must 
be  protected  from  blows,  pressure  and  scratcliing  and  the  arm  must 
not  be  used  too  strenuously.  The  best  covering  is  a  clean  hnen  sliirt 
sleeve  so  that  the  arm  is  cool  and  dry.  If  woolen  is  worn  a  clean  piece 
of  linen  or  silk  should  be  placed  inside  the  sleeve  over  the  vesicle.  Wet 
or  oily  dressings  soften  the  top  and  favor  rupturing  and  should  there- 
fore be  avoided.  Most  of  the  vaccine  shields  are  objectionable  as  they 
keep  the  wound  moist  and  warm  and  many  of  them  press  on  the  vesicle 
itself  or  interfere  with  the  circulation. 

If  there  is  a  marked  inflammation  of  the  arm  the  area  may  be  well 
powdered  or  if  tliis  does  not  succeed  cold  wet  dressings  either  of  salt 
solution  or  of  lead  water  may  be  used.  The  wet  dressing  should  be 
changed  every  fifteen  or  twenty  minutes,  and  as  soon  as  the  inflammatory 
reaction  sub.sides  the  arm  is  kept  cool  and  dry.  If  the  redness  returns, 
the  wet  dressing  may  again  be  used.    If  the  pustule  ruptures  the  dressing 


VACCINATION  353 

should  be  frequently  changed.  Lassar's  starch-zinc  paste  is  a  good 
dressing.  Scratched,  unclean  and  ulcerated  pustules  should  be  washed 
clean  with  warm  mild  antiseptic  solutions.  Blows  on  the  arm  or  too 
much  use  of  the  arm  may  cause  a  widespread  redness  which  may  be 
treated  with  applications  of  lead  water. 

The  German  vaccination  laws  regard  the  operation  as  a  surgical 
procedure  and  it  must  be  done  with  aseptic  instruments.  A  lancet 
pointed  needle  is  cheaper  than  a  lancet  and  a  platino-irichum  needle 
wliich  may  be  steriUzed  in  the  flame  after  using  is  especially  serviceable. 

The  vaccinated  indi\adual  must  be  washed  clean  and  be  kept  clean 
and  in  clean  clotliing  during  the  vaccination  period.     The  area  of  skin 

Fig.  75. 


Inoculation  points  in  a  calf  five  days  after  vaccination. 

is  usually  not  rendered  aseptic  but  merely  thoroughly  cleansed.  The 
vaccine  virus  should  be  kept  in  glass  tubes  in  the  dark  and  cool. 

In  Germany  the  vaccination  is  done  by  making  four  cuts  about 
1  cm.  long  and  deep  enough  so  that  the  scratch  looks  red  but  does  not 
bleed.  Into  these  a  small  quantity  of  the  virus  is  rubbed.  The  left 
arm  about  the  insertion  of  the  deltoid  is  the  place  usually  chosen.  For 
success  the  primary  vaccination  should  show  a  pustule,  for  the  subse- 
quent ones  a  papule  is  all  that  is  necessary.  Some  irregularities  in  the 
course  of  the  vaccination  may  be  observed. 

Since  the  introduction  of  animal  lymph  tlie  virus  is  no  longer  taken 
from  the  pustule  of  the  vaccinated  person  ;  only  under  exceptional 
circumstances  should  the  human  virus  be  used  and  then  the  health  of 

11—23 


§54  THE    DISEASES   OF    CHILDREN 

the  person  from  whom  it  is  taken  should  be  above  suspicion.  In  taking 
the  lymph,  all  but  one  pustule  should  be  punctured  ^\^th  a  lancet  and 
the  lymph  gathered  in  capillary  tubes,  which  should  be  sealed.  Only 
clean  lymph  from  an  uninflamed  pustule  should  be  used. 

In  Germany  the  original  virus  is  obtained  from  institutions  con- 
ducted by  the  state  and  in  supervised  private  laboratories.  Most  of  the 
lymph  is  obtained  by  the  so-called  "Retrovaccination."  That  is,  calves 
are  inoculated  with  virus  from  a  person  who  has  been  vaccinated  with 
the  virus  taken  from  a  calf.  If  vaccination  is  done  continuously  from 
calf  to  calf  the  pustule  becomes  poor  and  insufficient  but  the  strength 
of  the  virus  may  be  kept  up  by  retrovaccination.  Some  institutions 
keep  the  virus  going  by  using  animals  only  and  the  original  virus  is 
from  an  accidentally  discovered  cow-pox.  Injuries  from  vaccination 
are  rare  and  usually  arise  from  an  accidental  mixed  infection. 

SyphiHs  does  not  occur  in  using  animal  lymph  and  only  rarely 
occurred  under  the  old  conditions  of  arm  to  arm  vaccination.  Tuber- 
culosis, wliich  was  a  theoretic  possibility,  does  not  occur  in  using  animal 
virus.  Syphilis  does  not  occur  because  calves  do  not  take  it  and  tuber- 
culosis because  the  calves  are  slaughtered  after  the  lymph  is  taken 
and  the  virus  from  those  showing  tuberculosis  is  rejected. 


DIPHTHERIA 

BY 

Dr.  J.  TRUMPP,  of  Munich 

tr.\nslated  by 
Dr.  .\LFRED  hand,  Jr.,  Philadelphl^,  Pa. 


History. — Diphtheria  is  the  term  appHed  since  the  time  of  Bre- 
toniieau  and  Trousseau  to  a  disease  of  the  mucous  membranes  or  skin, 
occurring  epidemically  and  spreading  by  contagion,  and  characterized 
by  the  formation  of  a  membranous  deposit  with  general  symptoms  of 
specific  toxa>mia.  Physicians  had  long  been  acquainted  with  the  local 
manifestations  of  the  disease,  such  as  diphtheria  of  the  pharynx,  larynx, 
skin,  etc.,  but  the  relationship  between  these  different  types  of  the  dis- 
ease was  first  recognized  by  Bretonneau  and  his  pupils,  Velpeau  and 
Trousseau  (1821-28). 

The  exciting  cause  of  the  scourge,  a  peculiar  bacillus,  was  first  dis- 
covered in  diphtheritic  pseudomembrane  by  Klebs  in  the  year  1883, 
while  Loffler  in  1884  was  the  first  to  obtain  it  in  pure  culture  and  to 
demonstrate  its  pathogenic  action  in  the  lower  animals.  Although  these 
bacilli  could  be  found  in  almost  every  case  on  the  mucous  membrane 
attacked  by  the  diphtheria,  and  although  LofTler  succeeded  in  producing 
pseudomembranes  similar  to  the  diphtheritic  deposits  by  rubbing  ba- 
cilli into  the  injured  tracheal  mucosa  of  rabbits,  the  etiologic  importance 
of  the  bacilli  would  nevertheless  have  remained  in  doul)t  if  Roux  and 
Yersin  had  not  been  able  to  isolate  the  toxin  produced  by  the  Klebs- 
LofHer  bacillus  and  in  experiments  on  lower  animals  to  produce  with  it 
the  main  symptoms  of  diphtheria,  especially  the  characteristic  paraly- 
ses. The  discovery  of  the  exciting  cause  of  diphtheria  was  followed  ten 
years  later  by  the  discovery  and  introduction  of  a  specific  treatment  for 
the  disease,  von  Behring's  serum  therapy.  Received  at  first  with  scep- 
ticism, the  use  of  diphtheria  antitoxin  has  in  a  few  j'ears  conquered  the 
whole  world.  Diphtheria,  however,  still  belongs  in  the  class  of  dan- 
gerous diseases,  but  physicians  can  now  face  its  dangers  with  a  certain 
superior  calmness,  based  on  a  consciousness  of  a  clear  knowledge  of 
practically  all  the  variations  of  the  disease  and  on  the  possession  of  a 
remedy  with  a  sure  action  in  the  majority  of  cases. 

Epidemiology. — Diphtheria  is  seen  in  all  chmates  and  seasons  but 
is  more  prevalent  in  cold  countries  and  the  colder  months.  While  in 
the  earlier  centuries  it  apparently  always  occurred  in  more  or  less  sharply 

355 


356 


THE   DISEASES   OF   CHILDREN 


defined  epidemics,  by  the  middle  of  the  Nineteenth  Century  it  had  be- 
come pandemic,  owing  to  the  development  of  commerce  with  more  rapid 
means  of  transportation,  so  that  now  the  disease  practically  never  dies 
out  in  large  cities.    In  addition,  the  epidemics  have  shown  great  varia- 


FiG.  76. 


Diphtheria 


mortality   in  the  German  Empire, 
1877-1901. 


Fio.  77. 


i:;i 

W 

■ 

P 

Sffl 


-w- 


Infectious  diseases,  mortality  in  Ger- 
many, in  1900.  a,  typhoid  fever;  b,  scar- 
let fever;  c,  measles;  d,  whooping-cough; 
€,  diphtheria;  /,  tuberculosis. 


Fig.  78. 


\ 

V. 

^, 

V 

\ 

\ 

\  * 

u 

\ 

|\ 

lij 

II  «         fa  I 

Diphtheria  mortality  in  Munich,  1867-1900. 
Fig.  79. 


Total  mortality  of  diphtheria  in  Gratz 
1890-1903,  in  per  cent. 


tions  in  intensity,  in  the  course  of  a  single  year  as  well  as  over  longer 
periods.  In  the  preceding  century  the  mortality  from  diphtheria  in 
Germany  rose  steadily  until  it  reached  its  highest  point  of  122,000 
deaths  in  1886  (see  Fig.  76  and  "Century-curve  of  Mortality  from 
Diphtheria  in  Hamburg,"  Heubner's  Text  book.)  Since  then  it  has 
fallen  with  an  increasing  rate  of  decline  to  about  45,000  fatal  cases  in 


DIPHTHERIA  357 

1900  (Fig.  77).  Marked  differences  were  often  shown  by  the  differ- 
ent cities,  this  variation  in  each  locality  being  dependent  upon  the 
favorable  or  unfavorable  character  of  the  disease,  which  can  change 
from  year  to  j^car  in  any  place.  Figure  78  shows  the  diphtheria  mor- 
tality in  Munich,  the  maximum  being  passed  long  before  188G.  The 
chart  giving  the  number  of  deaths  does  not  show  so  decidedly  the  intro- 
duction of  the  antitoxin  as  does  the  fig.  so. 
chart  giving  the  percentage  of  the  fatal 
cases  (see  Fig.  79,  percentage  of  fatal 
cases  in  Gratz).  The  approximate  pro- 
portions of  the  favorable  and  severe 
forms  of  diphtheria  for  the  last  decade 
in  German  cities  is  given  in  Fig.  80. 

The  primary  localization  of  the 
disease  and  its  character,  as  well,  have 
changed  at  different  times.  We  now 
see  the  disease  beginning  almost  always 
in  the  pharynx,  and  other  initial  points 

arc    rather   rare.        In   the   first  half  of  the  Table  showinB  the  frequency  of  the  clinical 

forms  of  diphtheria  and  their  mortality. 

precedmg    century,    however,    primary 

diphtheria  of  the  skin  and  larynx  were  frequently  met  with;  the 
Saxony  physicians  before  1860  were  acquainted  only  with  croup  and 
looked  upon  pharyngeal  tliphtheria  as  a  great  rarity.  In  former  times 
also,  adults  were  very  much  more  frequently  attacked  by  the  disease 
than  is  the  case  to-day.  Nowadays,  diphtheria  is  seen,  with  few 
exceptions,  only  in  children.  The  greatest  morbidity  and  mortality 
occur  between  the  ages  of  two  and  five  years.  The  susceptibility  of 
infants  shows  a  great  increase  after  the  age  of  six  months.  From  the 
school  age  to  adult  life  the  incidence  of  the  disease  and  its  mortality 
show  a  steady  decline. 

ETIOLOGY 

THE    DIPHTHERIA    B.iCILLUS 

The  exciting  cause  of  diphtheria  is  a  bacterium  belonging  to  the 
group  of  the  actinomyccs,  whose  form,  growth  and  virulence  show 
marked  changes  according  to  the  culture-medium  and  the  age  of  the 
culture. 

Morphology. — Loffler  describes  it  as  a  non-motile,  straight  or 
slightly  curved  rod — with  an  average  length  of  that  of  the  tubercle  ba- 
cillus but  about  twice  as  broad — showing  rounded  or  often  swollen  ends, 
and  under  certain  conditions  appearing  stratified  or  granular  through 
an  irregular  absorption  of  the  stain  (thickening  of  the  chromatin,  ap- 
pearance of  degeneration,  involution  forms).     In  a  smear  preparation, 


358 


THE   DISEASES   OF   CHILDREN 


the  rods  lie  parallel  or  at  angles  to  each  other  in  groups  of  various 
sizes,  frequently  forming  letter-like  figures,  as  V,  W,  X  or  Y.  In  sec- 
tions of  the  pseudomembrane  they  are  seen  grouped  in  large  or  small 
nests  or  arranged  somewhat  like  a  fish's  tail.    In  addition  to  the  typical 


Fio.  81. 


Flo.  82. 


iv 


v. 


\y 


Diphtheria  baciUi  from  membrane 
taken  during  hfe.  Stained  with 
Ixiffler's  potassium  methylene  blue 
solution.  Washed  in  water.  From 
Vierordt's  Diagnosis. 


Diphtheria  baciUi  mixed  with  cocci. 
From  membrane  taken  during  life. 
From  Vierordt's  Diagnosis. 


V 


T 


Loffler  form  of  tlie  long  bacillus,  there  are  frequently  seen  young,  shorter 
forms  which  are  not  granular  and  are  wedge-shaped  or  cylindrical,  and 
also  giant-forms,  two  or  three  times  as  large,  with  bulbous  ends  which 
are  always  decidedly  granular  and  stratified,  so  that  they  are  easily 
Fig.  83.  mistaken    for    cocci.      Under    certain    ex- 

ceptional conditions  of  growth  there  are 
seen  thread-like  forms  which  may  or  may 
not  branch  (Abbott  and  Gildersleeve). 

Staining. — ^The  diphtheria  bacilU  take 
all  the  aniline  stains  readily,  and  resist 
Gram's  with  only  a  short  appHcation  of  the 
iodiniodide  of  potash  solution.  A  good 
picture  is  furnished  with  Loffler's  alkaline 
methylene  blue  solution  (30  c.c.  concen- 
trated alcoholic  solution  of  methylene 
blue,  60  c.c.  potassium  hydroxide  solution, 
1  :  10,000)  which  ought  to  be  made  fresh 
every  four  to  six  weeks.  When  stained 
with  Ziehl's  solution  or  aniline-gentian  violet,  the  bacilli  appear  more 
plump  through  a  swelling  of  the  plasma.  Double  staining  is  recom- 
mended to  bring  out  the  Babes-Ernst  granulations  and  especially  to 
difTerentiate  the  chromatin  from  the  enveloping  substance;   Roux  uses 


"il. 


N 


^  .  ■,  y 

Diphtheria  baciUi  (LofHer)  from 
bouillon  culture.  Stained  with  LofHer's 
methylene  blue.  Zei.ss  homog.  Immers. 
Va.  Ocular  4.  From  Vierordt's  Diagnosis. 


DIPHTHERIA  359 

dahlia  violet  and  methyl  green,  Neisser's  method  being  acetic  acid- 
methylene  blue,  crystal  violet  and  chrysoidin.  The  value  of  double 
staining  is  discussed  in  the  section  on  diagnosis. 

Growth. — The  diphtheria  bacillus  needs  for  its  growth  a  culture 
medium  with  shghtly  alkahne  reaction.  The  hmits  of  its  growth  lie 
between  19°-42°  C.  (66°-107°  F.),  the  most  favorable  temperature  being 
from  33°-37°  C.  (91°-98.6°  F.  ).  It  grows  most  lu.xuriantly  on  an 
albuminous  medium,  especially  blood-serum,  wliich  is  used  as  a  selec- 
tive medium;  for  the  bacillus  develops  on  this  more  rapidly  than  other 
bacteria  which  may  accompany  it.  From  eight  to  twelve  hours  after 
inoculation  a  growth  of  minute  colonies  is  seen  on  the  surface  of  the 
serum  looking  like  ropes  of  droplets  which  soon  become  confluent  and  in 
about  two  days  cover  the  culture  medium  with  a  thick  white  overgrowth, 
the  edges  of  which  are  scalloped.  In  bouillon  the  effect  is  first  to  give  an 
acid  reaction  and  after  not  less  than  eight  days  a  return  to  alkaline. 
The  bacilli  grow  lu.xuriantl}'  in  milk  without  curdling  it.  With  the 
development  of  an  acid  reaction  in  the  culture  medium,  the  growth  and 
the  formation  of  toxin  (Madsen)  lessen.  A  similar  result  follows  a  change 
of  temperature  to  that  above  or  below  the  limits  mentioned. 

The  sensibility  of  the  bacilli  to  thermic  influences  is  very  varied. 
Cold,  even  the  action  of  winter-temperatures  for  months,  is  well  borne 
(Abel),  but  in  the  opposite  direction,  death  soon  follows  the  action  of  a 
temperature  of  50°  C.  (122°  F.).  The  bacilli  are  just  as  sensitive  also  to 
the  action  of  certain  chemicals,  especially  those  used  ordinarily  in  disin- 
fection in  the  usual  strengths: — alcohol,  lysol,  phenol,  tincture  of  the 
chloride  of  iron,  chlorine  water,  corrosive  sublimate,  cyanide  of  mercury. 
Much  less  powerful  are  boric  acid  and  permanganate  of  potash,  but 
peroxide  of  hydrogen-  is  very  useful. 

Although  the  bacilli  resist  heat  and  chemicals  so  feebly,  their  re- 
sistance to  drying  is  in  the  inverse  ratio,  especially  if  they  arc  enclosed  in 
bits  of  membrane  and  are  not  exposed  to  diffused  daylight.  Positive 
cultures  have  been  obtained  after  weeks  and  months  from  toys  and 
books,  from  furniture,  dishes,  floors  and  walls  of  previously  infected 
dwellings,  especially  if  dark  and  damp.  It  is  noteworthy  that  the  bacilli 
are  able  to  endure  a  temperature  of  98°  C.  (208°  F.)  for  an  hour  in 
the  dried  pseudomembranes  (Roux  and  Yersin). 

The  diphtheria  bacilli  have  a  pathogenic  action  on  lower  animals 
only  when  introduced  artificially.  If  the  tracheal  mucosa  of  a  rabbit  is 
injured  by  traumatism  and  the  bacilli  injected,  the  animal  dies  with 
symptoms  similar  to  those  in  membranous  croup.  At  the  autopsy  a 
fibrinous,  hfemorrhagic  exudate  is  found  on  the  trachea.  The  mucosa 
around  the  site  of  the  inoculation  is  strongly  reddened  and  covered  with 
a  grayish  yellow,  thick,  tenacious  pseudomembrane,  from  which  the 
bacilli  can  be  cultivated.   Identical  appearances  follow  if,  instead  of  the 


360  THE    DISEASES   OF   CHILDREN 

bacilli,  a  germ-free  filtrate  of  a  culture  is  used  for  the  injection,  one  being 
chosen  which  does  not  cause  death  too  suddcnlj^  (Roux,  Roger  and 
Bayeux,  Trunipp  and  Ziegler).  Control-animals  recover  from  the  trau- 
matism very  promptly  and  the  mucosa  shows  only  small  reel  tleposits. 
While  the  animals  in  such  experiments  die  from  obstruction  of  the 
trachea,  when  the  bacilli  or  the  toxin  are  injected  subcutaneously  or 
intraperitoneally  the  animals  die  with  symptoms  of  general  intoxication 
and  if  the  course  is  prolonged,  paralyses  occur  which  resemble  the  post- 
diphtheritic paralyses  in  human  beings. 

The  typical  findings  are  fijjrinous  exudate  at  the  site  of  injection, 
inflammation  of  the  serous  membranes  and  hypersemia  of  the  adrenals. 
If  the  bacilli  are  injected  they  are  found  only  at  the  site  of  inoculation. 
Death  can  therefore  be  the  result  only  of  intoxication.  The  fact  that  the 
pseudome?7ibranes  which  follow  the  injection  of  bacilli  are  also  caused  by  an 
injection  of  the  germ-free  toxin  proves  that  their  development  is  to  be  attrib- 
uted to  the  action  of  the  poison. 

In  the  animals  which  survive  the  injections  there  is  found  an  in- 
creased resistance  to  the  action  of  further  doses  of  the  toxin,  but  this 
acquired  immunity  to  the  poison  docs  not  prevent  the  possibility  of 
diphtheria  baciUi  obtaining  a  foothold  and  multiplying  on  the  mucous 
membranes.  On  the  ability  to  transfer  from  animal  to  animal  and  from 
animal  to  man  this  condition  of  imnninity  rests  Behring's  serum  therapy. 

Not  all  of  the  Loffler  bacilli  are  pathogenic.  Their  virulence,  or  the 
abihty  of  their  protoplasm  to  produce  toxin  is  very  different  (but  not 
necessarily  so)  in  that  bacilli  from  mild  cases  are  less  virulent  than  those 
from  severe  cases.  The  results  of  animal  experiments  and  clinical  obser- 
vation often  harmonize,  but  not  always.  This  lack  of  harmony  is  prob- 
ably due  to  the  variations  in  the  living  material,  and  also  sometimes  to 
chance  in  the  experimentation.  The  bacilli  cultivated  from  one  case  do 
not  all  show  the  same  degree  of  power,  as  virulent  and  non-virulent  may 
be  found  side  by  side.  The  latter  either  show  all  the  characteristics 
typical  of  the  Loffler  bacillus,  or  vary  somewhat  in  their  morphological 
or  cultural  peculiarities,  which  has  led  Loffler,  Hoffmann-Wellenhof, 
Escherich  and  others  to  view  them  as  a  special  kind  of  bacilli,  the  pseudo- 
diphtheria  bacilli.  But  since  it  has  been  possible  by  a  passage  through 
animals,  whose  resistance  has  been  lowered  by  non-fatal  doses  of  diph- 
theria toxin,  to  change  typical  pseudodiphtheria  bacilli  into  highly  viru- 
lent diphtheria  bacilli,  typical  morphologically  as  well  as  culturally 
(Trumpp),  they  can  no  longer  be  considered  a  distinct  species. 

This  experiment  also  throws  interesting  light  on  the  activity  of 
other  bacteria  which  accompany  the  diphtheria  bacilli  in  the  so-called 
mixed  infections.  The  latter  are  looked  upon  mainly  as  secondary 
processes  in  which  pyogenic  cocci  and  bacteria  of  putrefaction  plaj'  the 
main  r61e.    We  cannot  yet  grant  that  they  enter  into  a  kind  of  symbiosis 


DIPHTHERIA  361 

with  the  bacilli  and  thereby  increase  the  virulence  of  the  latter  (Roux) 
but  rather  that  their  entrance  into  the  tissues  and  fluids  is  made  possi- 
ble by  the  activity  of  the  diphtheria  bacilli,  and  that  the  cocci  in  turn 
lower  the  resistance  of  the  organism  to  the  bacilli  or  their  toxin.  They 
can  be  looked  on  as  sharers  in  the  disease-process  only  when  they  are 
deep  in  the  pseudomcmbrane  or  in  the  submucous  tissues,  but  not  if 
they  arc  only  h'ing  on  the  pseudomembrane,  for  dozens  of  species  of 
streptococci  and  staphylococci  are  included  among  the  common  inhabi- 
tants of  the  mouth  and  are  found,  in  consequence,  also  in  the  superficial 
layers  of  the  false  membrane.  When  the  mixed  infection  is  streptococcal, 
the  symptoms  are  like  those  of  other  septic  processes.  According 
to  Bernheim,  blood-infection  is  not  always  necessar}',  as  the  process 
need  not  advance  further  than  an  intoxication,  the  streptococci 
remaining  in  the  false  membrane  while  their  toxins  are  absorbed.  The 
result  then  is  a  combined  action  of  the  toxins  of  the  diphtheria  bacilli 
and  streptococci. 

In  almost  all  cases  v/hich  show  the  clinical  course  of  diphtheria 
Loffler  bacilli  are  found  in  the  local  deposit.  As  a  rule  they  are  found  only 
in  the  necrotic  tissue  and  exudate,  in  the  lymph-spaces  and  the  regional 
lymph-nodes.  Instances  of  the  bacilli  in  the  blood  during  life  or  in  the 
blood  and  internal  organs  after  death  are  extremely  rare.  Some  time 
after  the  decline  of  the  attack,  the  bacilli  disappear  from  the  pharynx 
but  they  may  remain  for  weeks  and  months  capable  of  multiplying,  and 
in  a  virulent  state,  on  other  mucous  membranes,  in  the  nasal  passages, 
on  the  conjunctiva  or  the  vulva.  The  bacilli  are  spread  from  one  person 
to  another  by  contact  with  the  sick  and  convalescent  and  also  by  arti- 
cles and  food  infected  by  them  (milk).  It  is  now  fully  established  that 
virulent  Loffler  bacilli  can  settle  on  the  mucous  membranes  of  healthy 
persons  without  causing  symptoms  of  the  disease;  and  so  frequently 
have  bacilli  which  belong  beyond  doubt  to  the  family  of  Corynebacter- 
ium  diphtherias  been  found  in  the  nose  and  on  the  conjunctiva  of  chil- 
dren and  adults  who,  so  far  as  is  known,  have  not  been  in  contact  with 
diphtheria  patients,  that  the  diphtheria  bacillus  may  be  considered  al- 
most ubiquitous.  These  considerations  show  us  that  the  mere  presence 
of  the  bacilU  is  not  sufficient  for  the  development  of  the  disease. 

PREDISPOSITION 

For  the  development  of  diphtheria  it  seems  that  much  more  [than 
the  presence  of  the  diphtheria  bacillus]  is  necessary:  (1)  that  the  bacilli 
have  attained  a  certain  degree  of  virulence;  (2)  that  these  bacilli  be  borne 
in  large  numbers  to  the  mucous  membranes;  (3)  that  the  infected  mucous 
membrane  is  at  the  time  of  infection  in  a  condition  which  gives  a  footing 
to  the  bacilli  and  favors  their  increase,  which  condition  is  a  not  too  acid 
reaction  with  a  loosening  or  abrasion  of  the  epithelium;  (4)  that  the 


362  THE   DISEASES    OF   CHILDREN 

infected  individual  i.s  in  a  general  way  receptive,  having  neither 
sufficient  general  resistance  nor  inherited  nor  acquired  specific  im- 
munity, or  at  least  only  to  an  inadequate  degree. 

A  potential  predisposition  may  at  times  be  increased  or  lessened, 
or  it  may  first  make  itseK  known  when  the  general  vitality  is  lowered 
by  an  accidental  sickness  wdiich  in  itself  is  quite  insignificant. 

Little  that  is  positive  is  known  about  the  special  local  predisposi- 
tions. A  lowered  predisposition  may  be  reasonably  attributed  to  a 
mucosa  of  firm  texture,  and  in  infants  during  the  early  months  of  life 
to  the  acid  reaction  of  the  buccal  cavity,  especially.  An  increased 
tendency  may  possibly  be  found  among  individuals  with  hypertrophy 
of  the  lymphoid  structures  of  the  pharynx,  which  form  in  the  majority 
of  cases  the  starting  point  of  the  local  process.  In  the  same  way  all 
affections  which  produce  a  loosening  or  inflammation  of  the  pharyngeal 
mucous  membrane,  seem  to  favor  the  infection. 

Numerous  investigations  point  to  the  existence  of  an  antitoxic 
state  of  the  blood,  not  only  in  convalescents  from  diphtheria,  but  also  in 
cliildren,  even  up  to  the  eleventh  year,  who,  as  far  as  known,  have  never 
had  diphtheria.  The  transmission  of  an  immune  body  in  the  milk  of 
women  convalescent  from  diphtheria  to  the  infants  nursed  by  them  is 
doubtful  (Auden). 

In  general,  a  disposition  to  contract  diphtheria  is  present  in  rela- 
tively few  persons,  probably  because  of  congenital  immunity;  at  least 
the  figures  for  the  morbidity  appear  rather  low  in  proportion  to  the 
numerous  opportunities  for  infection.  The  very  frequent  occurrence  of 
the  disease  in  early  childhood  can  in  part  be  attributed  to  the  fact  that 
children  of  this  age  are  creeping  on  the  floor  and  putting  their  soiled  and 
infecteil  fingers  frequently  into  their  mouths  (Feer).  The  immunity  of 
adults  in  spite  of  equal  chances  for  infection  [members  of  the  same 
family]  is  not  fully  explained. 

In  the  majority  of  cases,  survival  of  an  attack  of  diphtheria  fur- 
nishes an  immunity  for  the  rest  of  the  individual's  life,  and  yet  instances 
of  second  and  third  attacks  are  not  rare  (according  to  Zucker  in  9  to 
13  per  cent,  of  all  cases). 

Pathogenesis. — Under  the  above-mentioned  conditions  the  diph- 
theria bacillus  may  act  in  a  specific  manner  on  the  human  system.  It 
settles  on  a  prechsposed  mucous  membrane,  especially  the  pharynx,  and 
multiphes  with  rapidity.  If  the  number  of  the  bacilU  and  the  toxin 
manufactured  by  them  are  sufficient,  for  wliich  a  varying  length  of  time 
of  from  two  to  seven  days  is  necessary,  probably  dependent  on  the 
difference  in  the  local  predisposition  and  on  the  amount  and  intensity  of 
the  infecting  virus,  then  there  arise  symptoms  of  a  local  process  followed 
later  by  those  of  a  general  intoxication. 

The  next  step  consists  in  certain  changes  in  the  mucous  membranes. 


DIPHTHERIA  363 

The  poisonous  metabolic  products  of  the  bacilli  set  up  coagulation  necro- 
sis of  the  epithelium,  which  furnishes  a  still  more  favorable  culture 
medium  for  the  germs  of  the  disease.  At  the  same  time  the  poisons  dif- 
fusing through  the  epithehum  set  up  a  decided  inflammation  of  the  lim- 
iting layers  of  the  mucosa.  The  blood  vessels  in  the  region  become 
dilated  and  engorged  and  following  the  injur)-  to  their  walls  they  pour 
out  rapidly  and  richly  an  exudate  of  serum  and  fibrinogenous  substance. 
The  fibrin  ferment  set  free  by  the  death  of  the  tissue-cells  causes  a  coag- 
ulation of  tliis  exudate  pressing  into  the  necrotic  epithehal  layers,  and 
through  constant  repetition  of  the  process  a  pseudomembrane  is  formed. 

So  long  as  only  the  superficial  blood  vessels  are  exposed  to  the  ac- 
tion of  the  poison,  the  exudative  process  is  hmited  to  the  epithelial  laj^er 
of  the  mucous  membrane.  The  false  membrane  lies  on  the  mucosa  and 
can  be  easily  removed.  But  when  the  vessels  of  the  submucosa  are  af- 
fected there  then  follows  fibrinous  exudation  in  the  subepithehal  layers 
also,  and  the  false  membrane  is  then  formed  intimate  icith  the  mucous 
membrane,  so  that  it  can  be  removed  only  with  difficulty,  its  removal 
causing  bleeding  (croupous  or  diphtheritic  process  in  the  anatomical 
sense). 

The  compression  of  the  vessels  by  the  fibrinous  exudate  and  the 
impeded  circulation  cause  in  addition  a  necrosis  of  the  affected  tissues 
so  that  after  the  spontaneous  removal  of  the  false  membrane  deep  ulcers 
are  left  behind.  If  gangrenous  processes  set  in,  changing  the  mucous 
membrane  into  an  offensive,  dirty,  hquefjang  mass  or  into  a  firmer 
blackish  crust,  there  may  then  occur  mdespread  destruction  of  the 
mucous  membrane  going  on  even  to  destruction  of  the  underhing  car- 
tilages. Mention  must  also  be  made  of  the  fact  that  degenerative  pro- 
cesses in  the  blood  vessels  occur  not  only  at  the  site  of  the  local  processes 
but  also  in  situations  far  removed,  points  of  election  being  the  lung, 
pleura  and  adrenals. 

The  lymph-nodes  near  the  local  manifestations  are  always  affected, 
and  even  the  remote  lymph-nodes,  though  in  a  lesser  degree.  They  are 
swollen  and,  in  severe  cases,  inflamed;  in  gangrenous  processes  a^dem- 
atous  infiltration  also  occurs  in  the  periglandular  connective  tissue. 

Sooner  or  later,  following  these  changes  there  develop  more  or  less 
severe  general  symptoms,  wliich  can  only  be  attributed  to  the  absorp- 
tion into  the  circulation  of  the  toxins  formed  at  the  site  of  invasion,  for 
the  diphtheria  bacilh,  because  of  their  demand  for  oxygen,  multiply 
only  on  the  superficial  layers  of  the  mucosa,  especially  of  the  respira- 
tory tract  (preference  for  cyhndrical  epithehum),  hardly  ever  pene- 
trating into  the  flmds  and  internal  organs;  when  tliis  exceptional!)' 
occurs,  they  very  quickly  die  out.  A  febrile  disturbance  follows,  and 
symptoms  of  degeneration  appear,  affecting  especially  the  heart,  the 
parenchymatous  organs  and  the  peripheral  nerves. 


364  THE    DISEASES   OF   CHILDREN 

The  clinical  picture  produced  by  the  diphtheria  bacilli  and  the 
germs  ordinarily  aiding  them,  shows  certain  fundamental  differences: 
(1)  according  to  the  location  of  the  invading  germs;  (2)  according  to 
the  behavior  of  the  attacked  mucosa  toward  the  invasion;  and  (3)  ac- 
cording to  the  quantity  and  quality  of  the  toxin,  on  the  one  hand,  and 
on  the  susceptibility  of  the  patient,  on  the  other. 

The  onset  and  course  of  the  disease  may  be  violent  or  gradual,  and 
sometimes  the  local,  in  others,  the  general,  symptoms  predominate. 

The  mucous  membrane  reacts  to  the  bacillary  irritation  often  with 
only  sHght  superficial  inflammatory  products,  in  other  cases  with  a 
penetrating  inflammation  and  profuse  fibrinous  exudation.  This  may 
happen  in  the  same  incUvidual,  the  nuicosa  in  different  places  showing 
a  var3ing  reaction  to  the  same  infection. 

The  general  symptoms  of  toxaemia  may  be  limited  to  a  moderate 
fever  of  brief  duration  with  a  transient  albuminuria,  or  it  may  comprise 
severest  disturbances  of  the  general  well-being,  \vith  marked  albumin- 
uria, affection  of  the  myocardium  and  i)aralyses.  Local  and  general 
predisposition  usually  go  parallel  to  each  other,  but  tliis  is  by  no  means 
always  the  case  (Escherich).  Thus,  severe  toxaemia  may  accompany 
very  sUght  local  deposits,  and,  on  the  other  hand,  the  general  condition 
may  be  practically  undisturbed  with  extensive  membrane-formation. 

The  local  process,  as  well  as  the  general  intoxication,  can  bring 
about  a  fatal  termination.  The  local  process  does  so  when  it  is  situated 
in  the  air-passages,  with  an  inflammation  of  such  liigh  degree  that  the 
swelling  of  the  soft  parts  and  the  pseudomembranous  formation  prevent 
the  entrance  of  air.  The  general  toxaemia  results  in  death  when  it  causes 
irreparable  injury  to  the  vital  organs,  especially  the  heart.  Finally, 
death  may  be  caused  by  infection. 

Natural  recovery  follows  the  action  of  non-specific  protective  bodies, 
already  present,  the  alexins  of  the  blood,  as  well  as  the  specific  reaction  of 
the  organism  by  which  the  effect  of  the  diphtheria  toxin  is  in  part  neutral- 
ized. The  loosening  of  the  pseudomembrane  appears  to  be  brought 
about  in  a  special  way  by  the  entrance  of  staphylococci  into  the  meshes 
of  the  fibrin-network,  through  wliich  they  are  scattered  extensively. 
Their  metaboHc  products,  in  a  chemotactic  way,  bring  out  great  num- 
bers of  leucocytes  to  act  as  phagocytes;  these,  by  their  death,  favor 
the  destruction  and  removal  of  the  membrane.  Then  the  fibrin  turned 
to  pus  or  fat  is  thrown  off  and  expectorated.  In  the  larynx  and  trachea 
the  deposit  is  more  quickl}'  removed  because  it  is  hfted  up  by  the  in- 
creased secretion  of  the  mucous  glands  of  the  membrana  propria,  and 
so  is  loosened  in  its  whole  extent.  The  heaUng  of  the  ulceration,  left 
after  the  false  membrane  has  been  shed  (rare  in  the  larynx  and  trachea), 
is  brought  about  by  the  prohferation  of  the  intact  epithelium  in  the 
vicinity,  which  gradually  grows  over  the  gap.     Very  deep  diphtheritic 


DIPHTHERIA  365 

ulcers  are  followed  by  permanent  loss  of  substance  with  scar-formation 
(healing  only  by  the  activity  of  the  subepithelial  connective  tissue). 

ANATOMY 

Mucous  Membrane. — The  mucous  membrane  afTectcd  by  diph- 
theria is  swollen,  oedematous,  strongly  injected,  often  hemorrhagic, 
while  the  pseudomembranes,  which  vary  in  extent  and  in  the  tenacity 
with  which  they  adhere  to  the  underlying  tissues,  show  all  variations  in 
color  from  black  to  white.  They  are  granular,  crumbling  and  soft,  or 
firm,  tough  and  elastic.  On  section  the  thin  membranes  are  merely  a 
cellular  fibrinous  infiltration  of  the  superficial  layer  of  epithelium.  In 
the  thick  membranes  different  layers  are  seen.  At  the  top  is  a  layer  of 
granular  detritus  in  which  are  found  diphtheria  bacilli  and  the  sapro- 
phytes ordinarily  present  on  the  mucosa.  Below  this  is  a  layer  of  fibrin 
with  very  close  filaments  containing  only  diphtheria  bacilli  and  remains 
of  the  epithelium  which  can  scarcely  be  recognized.  Next  comes  a  net- 
work of  fibrin  enclosing  leucocj^tes  and  more  or  less  altered  epithelial 
cells,  tlie  filaments  becoming  furtlier  apart  towards  the  mucosa.  In 
''croup"  only  the  superficial  layer  of  epithelium  is  necrotic,  the  layer  of 
fibrin  with  leucocytes  sprinkled  through  it  is  sharply  outlined  with  its 
lamellated  structure  against  the  stratum  proprium.  In  "diphtheria" 
the  membrane  and  necrosis  extend  to  the  submucosa,  fibrin-formation 
is  seen  in  the  follicles  of  the  glands  and  in  the  lymph-spaces,  in  the  limit- 
ing connective  tissue;  in  the  swollen  lymph-nodes  of  the  neighborhood 
and  here  and  there  in  the  l^lood  vessels.  At  the  boundarj^  of  the  ne- 
crotic tissue  lies  a  dense  wall  of  leucocytes  (see  Plates  21  and  22). 

The  lymph-nodes  are  swollen,  hypersemic,  hemorrhagic  and  show 
diffuse  or  circumscribed  necrosis  on  section.  At  times  the  periglandular 
tissue  is  infiltrated.  Microscopically  there  are  not  rarely  found  groups 
looking  like  miliary  tubercles  but  without  caseation  or  giant-cells.  The 
blood  vessels  of  the  lymph-nodes  may  be  occluded  with  thrombi. 

The  lungs  are  almost  always  affected.  They  may  be  involved  in 
every  part  from  the  bronchial  mucous  membrane  to  the  pleura.  The 
changes  may  be  simply  those  of  catarrh  or  more  frequently  broncho- 
pneumonia with  vicarious  emphysema.  The  pneumonia  maj'  follow  an 
extension  of  the  catarrhal  process,  or  it  may  have  its  starting-point  in 
the  formation  of  infarcts  in  the  blood  vessels.  Fibrinous,  hamiorrhagic 
or  serous  exudates  in  the  pleural  cavity  are  of  frequent  occurrence. 
Sometimes  latent  tuberculosis  of  the  lymph-nodes  becomes  lighted  up 
as  a  sequel.  In  addition  to  pneumococci  and  streptococci,  diphtheria 
bacilli  are  also  found  in  the  lesions. 

As  a  rule  the  heart  shows  hartUy  any  perceptible  changes  macro- 
scopically.  It  is  in  diastole  and  the  cavities  are  filled  with  firm,  fibrin- 
ous clots.     The  heart-muscle   is   pale,  grayish  brown   ami   on  section, 


366  THE   DISEASES   OF   CHILDREN 

whitish,  somewhat  oedematous  and  brittle.  In  almost  every  case  the 
microscope  shows  decided  changes  in  the  myocardium.  In  rapidly  fatal 
cases  there  is  only  decided  fatty  degeneration.  When  the  cases  run  a 
longer  course  the  changes  are  more  severe,  consisting  of  disappearance 
of  the  transverse  striations,  massing  together  of  fat  granules  (in  high 
degree  only  when  severe  anaemia  has  existed),  vacuole  formation,  with 
fragmentation  of  the  muscle  bundles.  In  18  cases  of  postdiphtheritic 
heart-paralyses  Eppinger  found  destruction  of  the  muscle  fibres  result- 
ing from  a  solution  of  the  cell  substance,  myolysis,  with  frequent  block- 
ing of  the  attendant  muscle-capillaries  bj'  destroyed  blood  corpuscles. 
The  changes  most  frequently  found  are  those  affecting  the  interstitial 
tissue  with  or  without  involvement  of  the  parenchyma  at  the  same 
time,  consisting  of  oedema  with  an  aggregation  of  lymphocytes  and 
fibroblasts.  As  a  result  of  degeneration  of  the  heart-muscle  and  con- 
nective tissue  overgrowth,  fibrous  myocarditis  may  develop.  In  the 
endocardium  primary  necrosis  is  not  rare  with  thrombus-formation  fol- 
lowing. In  the  vessels  of  the  heart,  as  in  the  other  organs  proliferation  of 
the  intima  sets  in  (see  Plate  22). 

Disturbances  of  the  kidneys  from  the  simplest  to  the  severest  forms 
are  found  in  all  cases.  The  kidneys  are  usually  somewhat  enlarged, 
pale,  with  the  cortex  thickened,  the  medullary  substance  showing  red- 
dish streaks,  and  the  organ  as  a  whole  firm,  tough,  glistening  and  moist. 
Microscopically  about  two-thirds  of  the  cases  show  exclusively  or  at 
least  principally  a  degeneration  of  the  epithelium,  especially  of  the  con- 
voluted tubules  and  the  descending  Henle's  loops.  For  the  most  part 
the  cells  are  non-granular,  swollen  and  containing  fat-droplets,  and  in 
many  places  they  are  so  completely  destroyed  that  only  a  structureless 
mass  remains  (Diphtheritic  parenchymatous  nephritis,  Plate  22).  Nu- 
merous hyalin  casts  are  seen  in  the  collecting  tubules  (Heubner).  Intersti- 
tial changes,  massing  of  cells  in  the  blood  vessels  and  infiltration  of  the 
connective  tissue  as  well  as  the  very  rare  involvement  of  the  glomeruli, 
are  found  almost  solely  in  older  children  after  a  protracted  course  of  the 
disease.  Exceptionally,  hjemorrhage  into  the  tubules  and  chronic  pro- 
cesses in  the  form  of  atrophy  arc  found  (Councilman,  Mallory  and  Pearce). 

The  liver  is  large,  glistening,  blood-red,  firm  and  moist,  with  the 
acini  visible,  and  on  microscopic  examination  shows  decided  paren- 
chymatous degeneration. 

The  splee7i  shows  nothing  macroscopically  except  swelling  of  the 
follicles.  Microscopically  there  are  seen  hyalin  degeneration  of  the 
arteries  and  foci  of  epithelioid  cells. 

The  peripheral  nerves  show  in  many  cases  fatty  degeneration  of  the 
medullary  sheath  with  sweUing  and  disappearance  of  the  axis-cylinder 
(degeneration  starting  from  the  medullary  sheath)  and  an  overgrowth 
of  connective  tissue. 


DIPHTHERIA  367 

Types  of  the  Disease. — According  to  the  location,  distinction  is 
made  between  diphtheria  of  the  pharynx,  nose,  larynx,  vulva,  con- 
junctiva and  of  the  skin,  or  wound-diphtheria.  The  most  frequent 
variety  is  diphtheria  of  the  pharynx,  which  usually  appears  in  a  local- 
ized, favorable  form,  but  it  may  show  a  progressive  character  with 
more  or  less  toxic  symptoms,  or  it  may  assume  a  highly  malignant 
gangrenous  type.  Ordinarily  a  case  shows  the  triad  of  symptoms, 
necrosis  of  the  mucous  membrane,  fibrinous  exudation  and  presence  of 
the  Lofller  bacilh.     Transitional  forms  are  often  seen. 

1.  PHARYNGEAL   DIPHTHERIA 
(o)  LOCALIZED  PHARYNGE.\L  DIPHTHERIA 

Onset. — At  the  start  subjective  symptoms  of  discomfort  are  so 
slight  that  it  is  usually  difHcult  to  fix  a  definite  onset  for  the  disease. 
The  children  feel  somewhat  tired,  they  are  disinclined  to  eat  and  to  play, 
they  are  sleepy,  the  voice  is  rather  hoarse  and  slightly  nasal  from  mod- 
erate occlusion  of  the  nostrils.  The  child  breathes  through  the  mouth 
and  the  respirations  are  visibly  accelerated.  Occasionally  the  onset  is 
very  sudden,  with  chill,  high  fever  and  headache.  An  unpleasant  odor 
to  the  breath  and  elevation  of  temperature  are  usually  the  first  symptoms 
that  bring  the  child  to  the  physician  and  although  the  child  has  not 
seemed  sick  enough  to  be  put  to  bed  it  looks  pale  and  tired.  The  cer- 
vical lymph-nodes,  especially  those  at  the  angle  of  the  jaw  and  sometimes 
also  the  submaxillary  nodes,  are  swollen  on  one  or  both  sides,  being 
hard  and  somewhat  movable.  An  irritating  discharge  flows  from  the 
nostrils.  The  pulse  and  respiration  are  accelerated,  and  the  first  sound 
of  the  heart  is  often  rather  impure.  The  temperature  is  usually  between 
38°  and  39°  C.  (100.4°  to  102.5°  F.)  and  is  rarely  higher.  Pain  in  the  neck 
and  discomfort  on  swallowing  are  sometimes  present  early.  On  inspec- 
tion of  the  mouth  and  pharynx  only  slight  evidences  of  inflammation 
are  seen.  The  tongue  is  dry  and  moderately  coated,  the  pharyngeal 
mucous  membrane  is  a  little  reddened  and  glistening  with  increased 
secretion,  the  pillars  and  tonsils  of  one  side,  rarely  both,  are  prominent. 
On  the  tonsil,  less  frequently  on  a  swelling  to  one  side  of  the  posterior 
pharyngeal  wall  is  seen  a  small,  shmy-looking  deposit  which,  after  wip- 
ing away  the  mucus,  is  found  to  be  a  pseudomcmbrane  adherent  to 
the  mucosa  and  without  sharplj'  defined  edges  (Plate  20).  In  a  still 
earlier  stage  the  appearance  is  that  of  a  web-like  etching  on  the  mucous 
membrane.  It  can  usualh'  be  loosened  without  injuring  the  underlying 
structures  and  if  it  is  rubbed  between  two  cover-slips,  the  firmness  of 
its  structure  can  be  appreciated.  On  staining  the  preparation,  fibrin  is 
found  with  Loffler  bacilli  aggregated  in  clumps,  in  company  with  the 
saprophytes  of  the  oral  cavity.  The  further  course  of  the  case  depends 
on  whether  or  not  the  specific  treatment  is  adopted. 


368 


THE   DISEASES   OF   CHILDREN 


If  the  antitoxin  is  injected  immediately,  the  spread  of  the  mem- 
brane ceases,  or  during  the  next  twenty-four  hours  it  extends  over  only 
the  immediate  surroundings  ;  new  deposits  of  fibrin,  ordinarily  only 
small  ones,  may  appear  during  this  time  on  various  parts  of  the  pharyn- 
geal or  oral  mucosa  as  a  result  of  the  action  of  the  germs  before  the 
administration  of  the  antitoxin.  With  a  rapid  fall  of  the  temperature 
and  pulse  to  the  normal,  the  pseudomembranes  undergo  liquefaction, 
they  become  sharply  circumscribed  and  are  either  thrown  off  in  flakes  or 
melt  away  more  slowly,  disappearing  entirely  by  the  end  of  the  third  day. 
Eight  days  after  the  onset  of  the  first  symptoms  the  children  feel  so 
completely  recovered  that  it  is  almost  impossible  to  keep  them  in  bed. 
Without  the  gracious  help  of  the  antitoxin  the  course  of  the  disease 
is  usually  very  much  more  protracted.  The  membrane  appears  on  sym- 
metrical parts  or  spreads  by  continuity 
until  it  may  finally  cover  like  a  velvet 
skin  both  sides  of  the  fauces,  the  uvula 
and  even  small  spots  on  the  posterior 
pharyngeal  wall.  It  then  remains  station- 
ary for  five  or  six  days  (Plates  20  and 
21).  Sometimes  the  nasal  passages  are 
also  affected  but  here  the  diphtheria  is 
seldom  so  intense  as  to  lead  to  the  for- 
mation of  false  membrane.  There  are  ca- 
tarrhal changes  with  a  profuse  discharge, 
sometimes  tliin,  sometimes  mucopurulent 
or  of  pure  pus,  and  the  voice  has  a  decided 
nasal  character.  One  nostril  is  more  ob- 
structed, as  a  rule,  than  the  other.  With 
the  spread  of  the  local  condition  the  general  condition  becomes  worse 
(but  to  tliis  there  are  exceptions)  with  pain  on  swallowing,  tenderness 
on  palpation  of  the  lymph-nodes,  with  increased  depression  and  total 
anorexia.  The  pulse  is  accelerated  in  ])roportion  to  the  fever  which  falls 
more  slowly  than  when  the  antitoxin  is  used.  Moderate  albuminuria 
is  found  in  some  cases.  The  entire  course  rarely  lasts  longer  than  a 
week,  but  convalescence  is  protracted.  The  prognosis  without  anti- 
toxin is  always  doubtful,  for  even  in  apparently  mild  cases  the  local 
condition  may  suddenly  spread  to  the  larynx,  or  severe  toxajmia  or 
secondary  infections  may  occur.  Postdiphtheritic  paralysis  is  also  met 
with  at  times,  even  when  the  case  has  not  been  one  of  great  severity. 

In  addition  to  tliis  wliich  is  the  ordinary  form  of  localized  diph- 
theria, rudimentary  forvis  are  seen  from  time  to  time  presenting  such 
shght  symptoms  climcally  that  the  diagnosis  is  suggested  by  the  simul- 
taneous occurrence  of  typical  diphtheria  in  other  members  of  the  house- 
hold and   the   suspicions   become  confirmed   by   bacteriological   study. 


Fig 

.  84. 

Day  oF disease 

31.  g-.  cT 

1. 

2- 

3 

+. 

5. 

6 

170  39  5 

120    150   39o 

A 

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yi 

90   35  b 

Moderately  severe  diphtheria  localized 
in  the  pharynx  with  typical  influence 
of  the  serum. 


DIPHTHERIA 


3G9 


In  some  of  these  cases  there  is  a  shght  pharyngeal  catarrh  with  a  mot- 
tled clouding  of  the  tonsillar  epithelium,  the  merest  suspicion  of  a  mem- 
brane. There  is  very 
moderate  fever  and 
complete  recovery  in 
a  few  days.  In  other 
cases  the  exudate  re- 
mains hmited  to  the 
crypts  of  the  tonsils 
and  the  appearance 
and  course  of  the  dis- 
ease show  great  simi- 
larity to  ordinary  fol- 
licular tonsillitis.  A 
further  discussion  of 
these  and  other  forms 

resembhng    tonsilUtis    and    stomatitis  will    be    found    in 
on  differential  diagnosis. 


Fir; 

.  81 

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Localized  pharyngeal  diphtheria  in   pre-antitoxin  days. 

the    section 


(6)  PROGRESSIVE   PHARYNGEAL  DIPHTHERIA 

The  onset  of  tliis  form  may,  like  the  preceding,  be  insidious  and  run 
a  rather  mild  course  with  moderately  severe  symptoms  of  general  tox- 
aemia, as  long  as  the  affection  of  the  larynx  is  not  so  great  as  to  offer 
considerable  obstruction  to  the  entrance  of  air.  In  the  majority  of  cases, 
however,  the  disease  sets  in  abruptlj^  and  with  severe  symptoms,  even 
v\ith  convulsions  in  very  young  children.  The  children  suddenly  feel 
very  sick,  sometimes  they  are  chilly,  there  is  severe  headache  with  gen- 
eral pains  in  the  body,  the  appetite  is  gone,  they  may  feel  nauseated  and 
they  sit  around  listless  and  dull  with  flushed,  swollen  features.  When 
put  to  bed  they  lie  rather  apathetic  with  a  temperature  of  39°-40°  C. 
(102°-104°  F.),  and  the  pulse  is  proportionately  accelerated  to  140  to 
160.  The  submaxillary  lymph-nodes  are  tender  and  swollen  to  the 
size  of  a  hazel-nut  while  those  at  the  angle  of  the  jaw  are  as  large  as  a 
walnut  and  sometimes  the  surrounding  region  is  prominent  with  a  boggy 
swelUng  due  to  a^dema  of  the  subcutaneous  connective  tissue.  The 
tongue  is  moist  and  slightly  coated  and  there  is  a  profuse  secretion  of 
tenacious  glistening  mucus  in  the  mouth  and  pharynx.  The  mucous 
membrane  of  the  mouth  is  bright  red  while  that  of  the  isthmus  and  of 
the  posterior  pharyngeal  wall  is  dark  or  streaked  mth  red.  The  faucial 
pillars,  the  tonsils,  the  uvula  and  the  lateral  roots  of  the  posterior 
pharyngeal  wall  are  swollen,  usually  more  on  one  side.  On  one  or  both 
tonsils  there  is  a  uniform,  mucous  exudate,  firmly  seated,  rarely  ap- 
pearing only  as  isolated  yello^\^sh  fibrinous  streaks  or  spots.  By  the 
end  of  the  first  or  the  beginning  of  the  second  day  of  the  disease  the 

11—24 


370 


THE   DISEASES   OF   CHILDREN 


cliildren  complain  of  burning  and  choking  in  the  throat  and  of  sharp 
pains  on  swallowing,  espcciall}'  when  the  mouth  is  empty.  Both  tonsils 
and  ultimately  the  uvula  and  parts  of  the  posterior  pharyngeal  wall  are 
now  seen  to  be  covered  with  a  grayish  white  membrane,  often  mottled 
and  either  smooth  or  lumpy  in  appearance.  In  the  course  of  the  next 
two  or  three  days  the  membrane  spreads,  finally  covering  the  whole 
pharynx,  the  anterior  and  posterior  pillars  of  the  fauces  and  advancing 
up  into  the  posterior  nares.  The  fever  and  the  severe  disturbances  of  the 
general  system  continue  or  even  increase  in  force.  The  odor  from  the 
mouth  becomes  unpleasant,  sweetish,  even  fetid.  The  sense  of  fulness 
in  the  throat  causes  dyspnoea.  The  voice  becomes  tlrick  and,  through 
fixation  of  the  faucial  pillars  and  occlusion  of  the  nasopharyngeal  space, 
decidedly  nasal.    Secretion  is  so  greatly  increased  in  the  nasal  passages 

Fig.  86. 


Day  of  disease 

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Progressive  diphtheria  terminating  in  recover^-.    Antitoxin,  intubation,  secondary  tracheotomy. 

as  to  occlude  them,  so  that  the  child  breathes  with  the  mouth  open. 
Otitis  is  not  a  rare  compHcation. 

The  kidneys  are  affected  in  almost  every  case.  After  about  the 
tlrird  day  an  abundant  sediment  is  found  consisting  of  many  small  epi- 
theUal  cells,  cylindroids  and  epithelial  casts;  from  the  first  to  the  third 
weeks  of  the  disease  there  is  albuminuria,  varying  in  amount  but  never 
being  very  great  (Heubner). 

As  a  rule  the  bowels  are  sluggish  even  far  into  convalescence. 
Examination  of  the  blood  shows  a  very  decided  leucocytosis  (L.  G. 
Simon).  In  cases  tending  to  recovery  the  proportion  of  the  lympho- 
cytes is  increased;  in  severe  cases  myelocytes  are  found  (Engel).  When 
antitoxin  is  injected,  the  advance  of  the  process  is  checked  in  the  great 
majority  of  cases.  The  fever  falls  rapidly  and  the  temperature  becomes 
normal  or  even  subnormal,  often  witliin  a  day.  At  the  same  time  the 
pulse-rate  falls  to  its  normal,  or  frequently  below,  the  pulse  becoming 
small  and  not  rarely  arrhythmical.     The  local  lesions  disappear.     The 


PLATE  20. 


DIPHTHERIA 


371 


general  strength  is  increased;  but  tlie  convalescence  is  nevertheless  pro- 
longed by  ana'niia  and  general  weakness.  Not  rarely  postdiphtheritic 
paralyses  appear,  with  secondary  infections,  especially  broncliitis  and 
pneumonia,  and  in  many  cases  there  is  the  threatening  danger  of  an  acute 
cardiac  failure,  which  may  also  appear  very 
unexpectedly  in  the  acute  stage. 

If  the  antitoxin  is  not  given  or  if  its  use 
is  delaj'ed  until  late,  the  local  process  may 
spread  by  continuity  or  it  may  leap  to 
different  spots,  advancing  to  the  anterior 
nares,  to  the  larynx  and  trachea,  while  in 
rarer  cases  the  niouth-ca\'ity  may  show  the 
false  membrane.  Following  these  severe  local 
changes,  which  may  sometimes  directly 
threaten  hfe,  symptoms  of  general  intoxica- 
tion set  in:  rapidly  developing  general  weak- 
ness, lowering  of  force  of  the  heart-beat,  e\d- 
denced  by  an  ominous  pallor  of  tlie  skin  and 
cyanosis  of  the  raucous  membranes;  coolness 
of  the  extremities  due  to  the  poor  quaLty 
of  the  blood-stream;  right  sided  or  general 
dilatation  of   the  heart   with   an   impure  first 

sound  at  the  mitral  orifice;  a  small,  arriiythmic  pulse,  becoming  slower, 
finally  thread-hke  and  imperceptible.  Death  occiu-s  toward  the  end  of 
the  second  week  with  collapse,  or  later  than  this  with  urtemia  or  dropsy. 

EXTEXSIOX    TO    THE    MOUTH-C.WITY 

"With  the  extension  to  the  mouth-cavity  there  appear  on  the  hard 
palate,  the  gums  and  the  lips  (Plate  21),  rarely  also  on  the  buccal 
mucosa,  thin,  milk-white  deposits  which  arc  at  first  isolated,  some  of 
them  later  coalescing:  these  false  membranes  thicken  and  become 
grayish  green  crusts  some  of  which  are  superficial  while  others  are  more 
deep-seated  and  can  be  removed  with  difficulty  and  not  without  bleed- 
ing and  loss  of  substance.  In  very  rare  instances  the  whole  oral  cavity 
is  found  lined  by  pseudomembranc.  In  all  these  cases  there  is  increased 
flow  of  saliva  with  an  offensive  odor  to  the  breath.  The  regional  lymph- 
nodes  are  enlarged  and  infiltrated. 


Fig 

.  87 

Day  of  disease 

%&.  s- 

3 

+ 

5^ 

6 

7 

1 

190   40  5 

j— 

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■ 

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60   100  36 0 

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90   35  i 

Progressive  diphtheria  with  fatal 
result.  Senun  treatment,  trache- 
otomy. 


EXTENSION  TO  THE  N.\.SAL  PASSAGES,  EUSTACHIAN  TUBE 
AND  MIDDLE  EAR 

The  false  membrane  of  a  pharyngeal  diphtheria  spreads  to  the 
nasal  passages  by  atlvancing  along  the  lateral  wall  of  the  pharynx  or 
else  by  coming  down  the  front  and  up  the  jjosterior  surface  of  the  soft 
palate,  more  rareh'  by  a  continuous  advance  from  the  posterior  pharyn- 


372  THE   DISEASES   OF   CHILDREN 


b 


geal  wall  up  along  the  base  of  the  skull.  High  fever  develops,  and  a 
sense  of  pressure  with  obstruction  of  the  nasal  passages.  Excessive 
secretion  at  first  serous,  later  bloody  and  containing  particles  of  mem- 
brane, finally  purulent,  flows  constantly  from  the  nostrils  over  the  lips; 
it  may  also  be  seen  in  the  pharynx,  coming  down  from  the  posterior 
nares.  The  skin  of  the  upper  lip  and  around  the  nostrils  is  red,  swollen, 
excoriated  and  covered  with  bloody  crusts,  which  may  disclose  a  thin 
membrane  as  they  fall  off.  The  necessary  breathing  through  the  mouth 
makes  the  tongue  and  lips  dry  and  fissured.  The  voice  is  thick  and 
palatal.  On  rhinoscopic  examination  the  mucous  membrane  is  seen  to  be 
very  red  and  swollen  and  on  the  septum  and  turbinates  there  are  gray- 
ish white  deposits,  isolated  or  presenting  a  frost-hke  appearance.  As  a 
result  of  the  confluence  of  these  spots  or  by  spreading  at  the  periphery 
the  membrane  enlarges  and  may  ultimately  form  a  thick,  fat-looking 
layer  covering  the  whole  mucosa  of  the  upper  air-passages  even  in  its 
deepest  folds,  filling  up  completely  the  pars  posterior  (the  pars  anterior 
is  rarely  attacked  alone  or  to  any  extent).  The  course  of  retrogression 
and  healing  occur  as  in  pharyngeal  diphtheria  and  take  about  the  same 
length  of  time.  Deep  ulceration  may  result  in  cicatricial  closures,  espe- 
cially synechia  of  the  septum  with  the  turbinates,  closure  of  the  mouths 
of  the  Eustachian  tubes,  partial  adhesions  of  the  soft  palate  with  the 
posterior  pharyngeal  wall  (W.  Anton).  Sometimes  the  disease  subsides 
to  a  chronic  form,  running  for  several  months  and  affecting  by  preference 
the  anterior  part  of  the  nasal  passages  (Concetti,  Monti). 

Involvement  of  the  nasal  passages  is  not  always  a  grave  compli- 
cation of  advancing  pharyngeal  diphtheria.  Sometimes,  however,  the 
nose  becomes  the  starting-point  of  a  gangrenous  diphtheria.  Swelling 
and  oedema  of  the  nose  occur  with  marked  pallor  and  a  characteristic 
shining  appearance  of  the  overlying  skin  (Oertcl),  involving  frequently 
the  cheeks  and  eyehds.  The  nasal  secretion  becomes  offensive  and  of 
bad  odor,  while  the  particles  of  membrane  in  it  are  of  a  blackish  hue  and 
fetid,  and  profuse  epistaxis  may  occur. 

In  about  three-fifths  of  all  cases  the  organs  of  hearing  become  af- 
fected. In  the  mildest  form  the  pharyngeal  mouth  of  the  Eustachian 
tube  is  closed.  The  tympanic  membrane  is  strongly  retracted  and  sub- 
jective sensations  of  hearing  arise.  In  other  cases  the  inflammation  and 
fibrinous  exudate  extend  exceptionally  through  the  tube  even  into  the 
tympanic  cavity  and  mastoid  cells  (Wendt,  Habermann). 

Diphtheritic  otitis,  which  may  begin  very  insidiously,  is  extremely 
painful.  It  is  accompanied  often  by  violent  headache  or  even  distur- 
bance of  consciousness,  and  it  causes  in  the  majority  of  cases  large  per- 
forations which  go  on  to  rapid  destruction  of  the  tympanic  membrane. 
Examination  with  the  speculum  shows  in  the  beginning  only  a  serous 
infiltration  of  the  drumhead,  obliteration  of  the  outlines  of  the  hammer; 


DIPHTHERIA  373 

later,  after  perforation,  firmly  seated  diphtheritic  false  membranes  are 
seen  deep  in  the  external  canal  or  in  the  tympanic  cavity. 

The  discharge  is  at  first  scanty  and  seropurulent,  but  after  separa- 
tion of  the  membranes  it  becomes  copious,  fetid  and  discolored  or  tinged 
with  blood.  The  course  of  such  a  middle  ear  suppuration  is  almost 
always  tedious  and  frccjuently  injurious  through  the  great  disturbances 
which  follow  the  destruction  of  the  hgaments  of  the  ossicles,  through 
caries  and  necrosis  and  the  extension  of  suppuration  to  the  labyrinth. 
Deafness  of  high  degree  often  persists  and  occasionally  total  loss  of 
hearing  (\Y.  Anton)  results. 

In  a  way  similar  to  the  involvement  of  the  Eustachian  tube,  the 
process  maj-  spread  to  the  lachrymal  canal  and  even  to  the  conjunctiva 
(see  conjunctival  diphtheria). 

EXTENSION    TO    THE    RESPIRATORY    ORGANS 

Extension  of  pharyngeal  diphtheria  to  the  larynx  and  trachea  is 
somewhat  less  frecjuent  (one-fourth  to  one-third  of  all  cases)  than  to  the 
nasal  cavities,  although  different  epidemics  may  show  great  variations. 
Occasionally  a  continuous  membrane  is  seen  extending  from  the  pharynx 
to  the  glottis;  but  as  a  rule,  the  larynx  becomes  involved  suddenly  on 
the  fourth  or  fifth  day.  The  primary  deposit  in  the  pharynx  may  not 
increase,  on  the  contrary  it  may  have  begun  to  lessen,  when  sudden  and 
unexpected  symptoms  of  croup  develop.  The  onset  may  be  stormy 
with  high  fever,  prostration,  decided  swelling  of  the  lymph-nodes,  and 
albuminuria,  or  it  may  be  more  gradual,  with  moderate  irregular  fever 
and  less  disturbance  of  the  general  condition. 

The  first  symptoms  are  tickling  or  pains  in  the  neck,  a  character- 
istic weakening  of  the  voice,  a  short,  rather  brassy  cough  and  a  more 
prolonged  character  to  the  breathing.  Decided  hoarseness  develops 
rapidly,  the  cough  becomes  hollow  and  barking  and  the  breathing  is 
slower,  more  labored  and  noisier. 

A  laryngoscopic  examination  shows  that  the  symptoms  are  mainly 
the  result  of  a  swelling  and  congestion  of  the  mucous  membrane,  and 
also  partly  due  to  the  beginning  of  fibrinous  exudation. 

As  the  case  progresses,  respiration  becomes  so  embarrassed  that 
the  patient  is  anxious,  as  shown  liy  the  attitude  and  expression.  Voice 
and  cough  become  almost  completely  silent.  Inspiration  and  expira- 
tion are  noisy,  lengthened  (especially  expiration)  and  extremely  laborecl. 
The  increasing  air-hunger  brings  into  play  all  the  voluntary  accessory 
muscles  of  respiration,  so  that  the  thorax  is  elevated  and  finalh'  is  almost 
constanth'  held  in  the  position  of  inspiration.  The  number  of  respira- 
tions is  somewhat  increased  to  twcntj'-eight  or  thiirty  to  the  minute. 
Nevertheless,  the  volume  of  air  entering  the  lungs  gradually  becomes 
insufficient  to  counterbalance  the  external  atmospheric  pressure  and  the 


374  THE    DISEASES   OF    CHILDREN 

parts  surrounding  the  thorax  begin  to  sink  in  more  and  more  with  each 
inspiration,  the  suprasternal  notch,  the  supraclavicular  fossse,  the  epi- 
gastrium and  in  rachitic  children  the  lower  ribs.  The  larj-nx  is  drawn 
down  with  inspiration. 

Although  the  amount  of  air  entering  with  each  inspiration  is  so 
small,  yet  it  suffices,  owing  to  the  incomplete  expiration,  to  distend  the 
lungs  gradually  and  to  bring  about  a  permanent  position  of  inspiration, 
so  that  the  lower  border  of  the  lungs  is  depressed.  The  condition  of  the 
patient  begins  to  be  wretched,  the  air-hunger  causes  excitement,  which 
becomes  greatly  increased  when  the  respiration  is  temporarily  com- 
pletely interrupted  by  loose  particles  of  membrane  or  mucus.  These 
attacks  of  asphyxia  usuall}-  last  for  only  a  fraction  of  a  minute,  during 
which  the  child  tosses  about  in  anxiety,  the  features  become  cyanosed, 
the  staring  ej^es  seem  to  start  out  of  the  head,  beads  of  cold  perspiration 
come  out  on  the  forehead,  from  time  to  time  the  crowing  inspiration 
can  be  heard  at  a  distance — and  death  follows  unless  the  obstruction  is 
coughed  up,  frequently  in  the  form  of  a  more  or  less  complete  fibrinous 
cast  of  the  windpipe  (Fig.  8S  shows  such  a  cast  which  extended  even 
to  the  bronchi  of  the  third  division).  After  the  attack  the  child  is  ex- 
hausted and  lies  bathed  in  perspiration;  the  breathing  is  better  but  not 
easy.  Renewed  formation  and  exfoliation  of  the  membrane  may  cause 
the  attacks  to  be  repeated  after  six  or  eight  hours. 

Laryngoscopic  examination  in  the  stage  of  stenosis  is  exceedingly 
difficult  and  one  should  hesitate  to  do  it.  If  feasible,  it  shows  a  spread 
of  the  fibrinous  exudate  with  islands  of  deposit,  some  of  them  confluent, 
in  the  interarytenoid  space  and  the  subglottic  region;  or,  in  extreme 
cases,  a  single,  uniform  wlutish  yellow  (macaroni-Uke)  membrane,  ex- 
tending from  the  posterior  surface  of  the  epiglottis  over  the  false  and 
true  vocal  cords  and  on  down  deep  into  the  trachea.  The  rima  glottichs 
looks  hke  a  narrow,  immovable  cleft,  whose  range  of  excursion  is  lim- 
ited particularly  by  the  drawing  together  of  the  arytenoid  cartilages  by 
fibrinous  bands  which  prevent  any  movement  of  abduction  (Piniazek) 
(Plate  20). 

All  of  these  symptoms  may  develop  in  twelve  to  twenty-four  hours, 
or  it  may  take  several  days.  If  there  is  no  spontaneous  chsappearance 
of  the  threatening  symptoms,  or  if  operative  interference  is  not  resorted 
to,  the  symptoms  of  carbon  dioxide  poisoning  come  on,  and  the  stage  of 
asphyxia  is  entered  (von  Rauchfuss). 

The  patient  grows  weaker  and  the  activity  of  the  accessory  muscles 
of  respiration  lessens.  The  restlessness  is  followed  b}'  an  ominous  calm. 
The  breathing  becomes  accelerated,  more  superficial  and  the  dyspncea 
seems  to  lessen,  but  the  deathly  pallor  of  the  skin  has  a  cyanotic  tint. 
The  features  are  drawn,  the  nose  is  pinched  and  prominent  and  the 
forehead  is  covered  with  a  cold  sweat.    The  extremities  grow  cool.    The 


DIPHTHERIA 


375 


pulse  may  have  the  paradoxus  type,  it  is  rapid,  thready  and  finally  not 
countable.  The  patient  falls  into  a  dreamy  doze  from  which  he  is  roused 
time  and  again  by  the  asphyxia,  starting  up  with  an  expression  of  great 
anxiety.  From  time  to  time  more  .severe  attacks  of  dyspnoea  set  in, 
without,  however,  causing  a  marked  reaction.  This  condition  persists 
or    tracheal    rattle  fig.  88. 

comes  on,  and  life  ebbs 
away  slowly  after  a 
more  or  less  protract- 
ed agonal  period,  witli 
advancing  paralysis  of 
the  centres  of  respira- 
tion and  circulation. 

The  course  is  not 
so  severe  in  all  cases. 
Some  patients  have 
only  aphonia  and  mod- 
erate dyspnoea,  but 
these  may  persist  for 
weeks  or  exceptionally 
for  months.  Even  the 
severe  cases  may  re- 
cover (about  one- 
sixth)  from  the  stage 
of  stenosis,  by  an  in- 
crease in  the  mucous 
secretion  tending  to 
dislodge  the  mem- 
brane. Like  the  local 
symptoms,  the  symp- 
toms of  specific  gene- 
ral intoxication  also 
show  great  variations. 
In  the  advanced  stage 
of  the  disease  it 
is  difficult  to  sepa- 
rate the  action  of  the 
diphtheria-to.xin    from   that  of  the  carbon  dioxide  intoxication. 

The  successful  outcome  of  operative  interference  depends  first  on  how 
far  the  local  process  has  advanced  in.  the  air-passages;  and  next,  on  the 
degree  of  general  intoxication,  especially  on  the  extent  to  which  the 
heart  is  capable  of  doing  its  work, — tliis  influencing  in  no  small  degree 
the  next  important  element,  the  disposition  of  the  deeper  air-passages 
to  a  primary  diphtheritic  infection  or  to  a  secondary  infection   with 


Fibrinous  exudate  in  the  lar>'nx.  trachea  and  bronchial  tree  extend 
iuK  to  bronchi  of  the  third  degree.  Denial  view,  showing  the  folds  of 
the  membrane  sniuotheti  out. 


376  THE   DISEASES   OF   CHILDREN 

pneumobacteria  (Heubner).  If  the  bronchial  tree  is  already  involved 
at  the  time  of  operation,  unless  the  fibrinous  exudate  becomes  melted 
away  by  prompt  administration  of  the  antitoxin,  the  outlook  for  recov- 
ery is  very  dark  and  the  operation  is  not  followed  by  improvement  in 
oxygenation,  the  expectoration  through  the  tracheal  cannula  is  weak, 
respiration  remains  superficial  and  rapid,  cyanosis  and  depression  in- 
crease and  death  follows  in  a  few  hours  or  days.  If  the  process  has  been 
limited  to  the  larynx  and  trachea,  the  symptoms  of  carbonic  acid  intox- 
ication disappear  after  the  operation  and  there  remain  only  the  effects 
of  the  toxin.  The  outcome  is  then  practically  dependent  on  the  time  of 
administration  of  the  antitoxin. 

Sometimes,  in  about  half  of  the  cases  not  treated  with  antitoxin, 
after  the  temporary  success  of  the  operation  and  even  with  a  falling 
temperature,  there  comes  a  turn  for  the  worse  due  to  a  descent  of  the 
fibrinous  inflammation  to  the  bronchial  tree,  involving  in  some  cases 
the  smallest  bronchi.  Its  onset  is  marked  by  an  elevation  of  temperature. 
Respiration  becomes  more  superficial  and  accelerated,  forty  to  eighty 
to  the  minute.  Inspiratory  recession  is  seen  at  times.  The  breath- 
sounds  over  the  lower  lobes  are  weak  or  inaudible,  over  the  upper  lobes 
they  are  whistUng,  sibilant,  coarse  and  accompanied  by  occasional 
rales.  The  further  the  process  advances,  the  greater  is  the  area  of 
the  lung  shut  off  from  respiration,  increasing  by  so  much  the  more  the 
carbon  dioxide  poisoning  to  which  the  patient  ordinarily  succumbs 
in  a  day  or  two. 

Far  more  frequently  the  fibrinous  process  stops  at  the  bifurcation 
of  the  trachea  and  the  bronchial  tree  is  subject  only  to  catarrhal  inflam- 
matory prox'esses,  bronchitis,  capillary  broncliitis,  lobular  and  pseudo- 
lobar  pneumonia.  These  are  caused  either  by  a  diphtheritic  infection, 
with  the  bacilh  demonstrable  in  the  affected  parts,  or  by  a  secondary, 
non-specific  infection  with  pneumobacteria  or  the  pyogenic  cocci.  These 
latter  infections  are  rendered  possible  through  the  resistance  of  the  or- 
ganism being  lowered  by  the  general  weakness  and  the  diphtheritic 
toxjemia,  and  are  favored  by  the  lessened  expectoration  with  stasis  of 
the  mucus. 

As  in  fibrinous  bronchitis,  these  processes  are  accompanied  by  in- 
creased fever  which  continues  rather  high  and  with  only  shght  remissions 
throughout  the  duration  of  the  illness.  The  breathing  again  becomes 
superficial  and  accelerated  and,  hke  the  cough,  is  painful.  Dyspnoea 
and  carbon  dioxide  poisoning  are  marked  in  the  severe  cases,  hke 
descending  croup.  Auscultation  and  percussion  give  no  special  signs. 
After  a  prehminary  crdema  of  the  lungs,  death  follows  in  collapse, 
or  else  there  is  a  protracted  course  lasting  long  after  the  primary 
diphtheritic  infection  is  overcome. 

Sometimes  serous  or  purulent  pleurisy  may  arise  to  complicate  the 


PLATE  21. 


a.  Diplitlieria  of  tonsils, 
6.  Diphtheria  of  the  lips. 

c.  Diphtheria  of  the  uvula.     The  epitlielium  is  for  tlie  most  part  necrotic,  tlie  cell-nuclei  swollen,  and  the 
cell-borders  no  longer  recognized.     The  vessels  are  enlar^eil   and  partly  hlled  with  tibrin. 


DIPHTHERIA  377 

conditions.  In  other  cases  the  diphtheritic  infection  produces  a  dark, 
haemorrhagic,  infarct-hke  infiltration  of  the  lung.  In  sucli  cases  there 
are  found  small,  circumscribed  areas  of  dulness  over  which  bronchial 
breathing  and  scattered  rales  are  heard.  If  the  lung  parenchyma 
becomes  broken  down,  cavernous  breathing  and  bubbling  rales  are 
heard,  followed  by  septic  fever,  a  putrid  odor  to  the  breath,  and  death. 
Another  possible  affection  of  the  lung,  which  is  also  accompanied  by 
very  high  fever  and  symptoms  of  widespread  pulmonary  involvement, 
is  caused  by  the  aspiration  of  foreign  bodies  such  as  particles  of  food. 
Tliis  is  seen  especially  in  operative  cases  and  has  as  sequels  abscess, 
gangrene  or  empyema. 

(c)  1VLA.LIGNAXT  PHARYNGEAL  DIPHTHERIA,  DIPHTHERIA  GRAVISSilA 

The  underlying  cause  of  tliis  most  severe  form  of  diphtheria  is 
either  an  extraordinary  virulence  of  the  baciUi  (with  the  combined 
action  of  aUied  streptococci  or  putrefactive  bacteria)  or  a  high  degree  of 
indi\Tidual  susceptibilit}'. 

It  rarely  appears  in  children  under  four  years  of  age,  and  its  fre- 
quency varies  gi-eatly  according  to  the  type  of  the  epidemic,  the  average 
being  from  ten  to  eleven  per  cent,  of  all  cases.  It  is  characterized 
by  widespread  and  deep-seated  lesions  of  the  mucous  membrane  often 
with  putrefactive  processes,  localized  in  many  places  (pharj-nx,  nose, 
mouth),  and  with  very  severe  toxic  symptoms.  It  may  be  secoudarj' 
to  a  localized  or  progressive  diphtheria,  or  it  may  be  primary  from 
the  start. 

In  the  former  instance  its  onset  is  more  insidious,  the  temperature 
is  hardly  raised,  while  the  threatened  danger  makes  itself  manifest  first 
in  a  rapid  and  profound  anaemia  and  decided  h^mph-node  enlargement. 

The  primary  form  sets  in  with  incredible  swiftness  and  violence. 
So  sudden  is  it  that  in  a  brief  space  of  time  all  resistance  is  battered  down. 
The  first  symptoms  are  fever  and  vomiting  with  tenderness  on  pressure 
in  the  epigastrium.  The  patients  are  greatly  excited,  sleepless,  occa- 
sionally slightly  delirious,  and  they  soon  become  prostrated  and  apa- 
thetic. Small  red  spots  appear  on  the  cool,  dvisky  skin.  The  features 
are  bloated  or  very  sharply  drawn  and  the  eyes  are  dull.  From  the  open 
mouth  or  the  red  and  swollen  nostrils  flows  blood  or  blood-stained  serum 
containing  particles  of  foul-smelling  membrane,  which  excoriate  the 
nares  and  lips.  The  air  of  the  room  is  laden  with  a  sweetish,  lime-like, 
cadaveric  fetor  from  the  breath  of  the  child. 

The  neck  is  tremendously  swollen  by  an  enlargement  of  the  lymph- 
nodes  and  oedema  of  the  periglandular  connective  tissue,  which  makes 
the  lateral  contour  of  the  neck  stand  out  prominently. 

Even  at  the  first  inspection  of  the  pharynx  the  soft  parts  are  found 
excessively  swollen  and  the  tonsils  at  times  so  greatlj'  enlarged  as  to 


378 


THE   DISEASES   OF   CHILDREN 


push  the  elongated  uvula  backwards  or  forwards.  Tonsils,  uvula,  pillars 
palate,  posterior  pharyngeal  wall  and  not  rarely  the  soft  palate  are 
covered  with  a  slimy,  grayish  yellow  or  blackish  membrane  dotted  with 
points  of  haemorrhage.  The  swollen  mucosa  in  its  uncovered  parts  shows 
intense  redness  and  isolated  areas  of  bleeding.  The  tongue  is  coated 
heavily  with  a  brown  or  blackish  slimy  deposit.  The  secretion  of  mucus 
is  greatly  increased.  Removal  of  the  membrane  in  the  pharynx  causes 
bleeding  and  loss  of  tissue;  it  is  usually  of  mushy  consistency  and  poor 
in  fibrin,  but  in  a  few  cases  it  is  tough  and  gristly  from  a  great  amount 
of  fibrin.  It  contains  many  cellular  elements,  diphtheria  bacilli  and  in 
almost  all  cases  streptococci,  more  rarely  staphylococci  or  colon  bacilli 
(Bernheim). 

The  temperature  may  remain  persistently  high  or  only  slightly  ele- 
vated, but  as  a  rule  it  falls  by  the  second  day  to  or  below  normal.     In 

other  respects  the  severity  of  the  picture  re- 
mains unchanged.  The  patients  remain  apa- 
thetic and  motionless  and  scarcely  pay  atten- 
tion to  the  most  urgent  demands.  Food  and 
liquids  are  pushed  aside,  from  dread  of  the 
pain  of  swallowing.  Even  in  willing  and 
rational  children  feeding  is  accomplished  with 
difficulty  because  of  the  excessive  swelling  of 
the  soft  parts  of  the  pharynx,  and  the  early 
development  of  paralysis.  The  speech  is 
unintelligible. 

The  swelling  in  the  neck  is  often  so  great 
that  the  head  is  held  stiffly  backward — Angina 
Ludovici.  The  pulse  is  very  rapid,  small 
and    compressible. 

Albumin  is  almost  always  present  in  the 
scanty  urine,  but  the  amount  does  not  accord 
with  the  severity  of  the  case.  As  a  rule  the  albumin  content  is  marked 
but  only  reaches  or  exceeds  two  per  mille  in  the  severest  forms  (Marfan). 
In  the  majority  of  cases  the  pharynx  becomes  clear — with  the  use 
of  antitoxin — in  about  eight  days.  Most  cases  show  more  or  less  deep 
ulcers  which  heal  slowly  with  scar  formation.  The  lymph-nodes  subside 
and  the  patient  enters  on  a  long  and  tedious  convalescence.  Marked 
weakness,  anaemia,  slowing  of  the  pulse,  arrhythmia  and  albuminuria 
may  persist  for  a  long  time.  Postdiphtheritic  paralysis  occurs  in  almost 
every  case. 

At  any  time  an  unfavorable  turn  may  come  in  the  course  of  the  dis- 
ease. Anajmia  advances  to  an  intense  degree,  with  great  general  weak- 
ness. The  pulse  becomes  thready,  extremely  rapid  and  arrhythmic. 
The  developing  heart-weakness  causes  signs  of  stasis  in  enlargement  of 


Fig.  89. 

Day  of  disease 

%&.  3- 

2 

3      4- 

5 

146  4(1.5 

no  180  40  0 

170  39  5 

. 

120    160   39 0 

Y 

~T 

H- 

_ 

150   385 

11 

nr 

V 

— 

li 

1  \ 

100   140  38o 

tf 

1  \ 

II  3 

130  37  5 

80  120  37o 

110  36  5 

60  100  36« 

SO  35  5 

Diphtheria  pravissima.  Most 
severe  form,  with  sudden  heart 
failure. 


DIPHTHERIA 


379 


the  liver  and  spleen,  with  dilatation  of  the  right  heart  and  at  times  of 
the  left  also.  The  apex-beat  is  diffuse  and  almost  imperceptible,  and 
the  sounds  are  weak,  especially  the  first  which  may  be  impure.  The 
pulse  finally  can  scarcely  be  felt.  The  weakness  of  the  patient  is  so  great 
that  dissolution  seems  imminent.  Towards  the  end  of  the  first  week  or 
the  beginning  of  the  second,  with  an  elevation  of  temperature,  vomiting 
sets  in,  a  certain  precursor  of  death.  The  pulse  falls  to  sixty  or  forty 
beats  per  minute,  and  the  end  comes  about  the  tenth  day,  sometimes 
earlier,  sometimes  later,  being  immediately  preceded  by  suddenly  de- 
veloping dyspna?a  of  high  degree,  cyanosis  and  an  expression  of  great 
anxiety. 

If  the  disease  runs  a  less  violent  course,  the  cervical  lymph-nodes 
may  suppurate  and  the  middle  ear  may  become  involved  by  extension  of 
inflammation  to  the  Eustachian  tube. 

The  larynx  and  trachea  are  not 
affected  in  black  diphtheria,  as  a 
rule,  or,  if  so,  marked  stenosis  rarely 
occurs.  In  such  cases  the  mucous 
membrane  is  deeply  reddened  with 
isolated  haemorrhages  and  dotted 
with  small  patches  of  false  membrane. 

In  some  epidemics,  however,  a 
considerable  exception  is  found  to 
this  rule,  amounting  to  twenty  per 
cent,  of  the  cases  of  malignant  diph- 
theria (Marfan).  In  these  cases  the 
local  process  advances  in  full  inten- 
sity to  the  larynx,  trachea  and  even  the  bronchi,  with  such  rapidity 
that  even  in  spite  of  early  treatment  by  antitoxin  and  operation,  the 
majority  of  cases  succumb  in  from  one  to  three  days  with  obstruction 
and  intoxication. 

Not  less  dangerous  but  running  a  somewhat  longer  course  is  the 
hccmorrhagic  form  of  malignant  diphtheria,  which  is  seen  in  about  twenty 
per  cent.  (Marfan).  Profuse  hicmorrhagcs  occur  from  the  nose,  mouth 
and  pharynx  which  can  with  difficulty  be  controlled.  There  are  also 
bleedings  in  the  stomach,  intestines  and  urinary  tract.  In  the  dusky 
skin  there  appear  numerous  spontaneous,  bluish  red,  green  or  black 
ecchymoses,  or,  on  very  slight  trauma,  larger  hirmorrhagcs.  On  the  ex- 
tensor surfaces  of  the  knees  and  elbows  many  cases  show  an  eruption 
like  that  of  scarlet  fever  (Marfan).  Vomiting  and  malodorous  diarrhoea 
contribute  to  a  state  of  great  discomfort.  With  a  profound  an£emia,  a 
progressive  weakness  of  the  heart  which  nothing  can  check,  a  falling 
temperature  and  a  failing  pulse,  death  occurs  after  a  few  hours  or  days, 
in  coma  or  convulsions,  or  with  the  signs  of  myocarditis  with  or  without 


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Malignant    diphtheria.     Protracted  form;   death 
from  heart  failure. 


380  THE   DISEASES   OF   CHILDREN 

cardiac  thrombosis.  In  more  protracted  cases  pneumonia  or  nephritis 
or  a  general  septic  state  may  develop. 

In  addition  to  these  types  there  may  be  more  fulminating  or  more 
protracted  forms  of  malignant  diphtheria. 

In  the  very  rare  hypertoxic  form,  as  in  cholera  sicca,  the  general 
intoxication  gains  the  upper  hand  so  quickly  that  death  occurs  in 
twenty-four  hours,  before  typical  local  changes  have  time  to  develop. 
The  general  symptoms,  which  appear  sudtlenly,  are  heart  failure, 
cyanosis  and  unconsciousness.  The  tonsils  are  seen  to  be  moderately 
swollen,  glistening,  red  and  as  if  covered  with  a  delicate  hoar-frost 
(Escherich). 

In  the  milder  forms  the  local  process  is  found  less  extensive,  or  only 
on  one  side,  with  less  of  a  tendency  to  necrosis.  The  accompanying 
phenomena  are  correspondingly  mild.  Because  the  course  is  more 
protracted,  there  is  time  for  the  development  of  the  sequels  of  the 
diphtheritic  to.xsmia  and  also  for  the  appearance  of  the  so-called 
serum  disease.  Secondary  infections  with  pyogenic  cocci  also  occur  in 
the  majority  of  cases;  purulent  inflammation  of  the  middle  ear,  the 
glands,  the  joints,  the  bones  and  the  serous  membranes  are  possibilities. 

The  majority  of  these  cases  are  saved  by  the  timely  administration 
of  antitoxin. 

2.   PRIMARY  NASAL  DIPHTHERIA,  DIPHTHERIA  OF  NURSLINGS 
AND  MEMBRANOUS   RHINITIS 

The  most  frequent  site  for  primary  diphtheria,  next  to  the  pharynx, 
is  in  the  nasal  cavities.  The  fibrinous  exudate  may  remain  limited  to 
the  nose  or  it  may  spread  through  the  posterior  nares  to  the  pharynx 
and  mouth,  or  passing  over  the  pharA'nx  it  may  leap  to  the  air-passages; 
in  rare  cases  it  may  extend  up  through  the  lachrymal  canals  to  the 
conjunctiva. 

It  is  likely  in  this,  as  in  pharyngeal  diphtheria,  that  the  lymphatic 
ring  of  the  pharynx  is  the  portal  of  Infection  for  the  diphtheria  bacillus, 
and  that  for  some  special  reasons  not  the  faucial  but  the  pharyngeal  ton- 
sil is  the  starting  point  of  the  process.  This  seems  to  be  the  case  espe- 
cially with  nurslings  in  whom  the  acid  reaction  of  the  oral  cavity  acts 
to  inhibit  the  growth  of  the  diphtheria  bacilli.  (The  fundamental  cause 
for  the  extremely  rare  cases  of  pharyngeal  diphtheria  in  the  newborn 
may  be  traumatism  of  the  oral  and  pharyngeal  mucosa  and  artificial 
inoculation  by  the  infected  finger  of  the  accoucheur. — Christcanu  and 
Bruckner). 

Primary  nasal  diphtheria  begins  ■nith  the  symptoms  of  a  marked 
coryza  with  fever,  a  feeling  of  heat  and  fulness  in  the  head,  and  of  dry- 
ness in  the  throat,  with  obstruction  of  the  nostrils,  earache  and  swelUng 
of  the  lymph-nodes  in  the  floor  of  the  mouth. 


DIPHTHERIA  381 

The  pharynx  is  dry  and  reddened  in  spots.  The  nasal  mucosa  is 
reddened  and  greatly  swollen,  discharging  an  abundant,  watery,  sero- 
mucus,  which  is  sometimes  bloody. 

After  a  day  or  two,  with  an  increase  of  fever,  the  fibrinous  exudate 
appears,  first  as  small,  isolated,  grayish  spots  which  soon  coalesce  to 
form  a  thick,  yellow  or  greenish  deposit,  which  may  become  brown 
from  extravasation  of  l)lood.  The  first  deposits  are  found  especially  on 
the  choana^  and  the  mouths  of  the  Eustachian  tubes  (W.  Anton).  Dur- 
ing the  whole  course  the  membrane  may  remain  Umited  to  the  naso- 
pharynx, but  cases  are  seen  in  which  the  brunt  of  the  attack  is  borne 
mainly  or  wholly  by  the  anterior  part  of  the  nasal  passages.  In  other 
respects  the  development  and  course  are  like  those  of  secondary  nasal 
diphtheria,  with  the  exception  that  secondary  complications  are  more 
frequent  in  this  form. 

Mention  should  be  made  of  an  appearance  of  pseudo-erysipelas  as 
described  by  Monti  and  Escherich,  starting  at  the  anterior  nares  and 
spreading  along  the  bridge  of  the  nose  up  to  the  forehead. 

If  there  is  not  transition  to  the  chronic  form,  recovery  occurs  in 
eight  or  ten  days  in  those  cases  which  are  not  progressive  or  which  do 
not  develop  complications.  The  exudate  becomes  limited  and  is  sepa- 
rated from  the  basal  membrane  by  an  increased  secretion  of  mucus 
which  becomes  admixed  with  the  purulent  discharge.  According  to  the 
extent  of  the  necrosis,  recovery  occurs  with  or  without  scarring. 

Some  peculiarities  are  seen  in  primary  nasal  diphtheria  in  the  new- 
born and  in  infants.  At  the  start  there  are  only  symptoms  of  a  decided 
coryza  :  a  brief  elevation  of  temperature  with  a  profuse,  watery  dis- 
charge from  the  nostrils;  a  high  degree  of  sweUing  of  the  nasal  mucosse, 
making  breathing  difficult  with  a  gurgling  sound,  while  it  is  hard  for 
the  infant  to  nurse,  owing  to  the  obstructed  respiration;  apathy  and 
stupor  follow  as  a  result  of  the  lessened  aeration  in  the  lungs,  with  the 
attendant  carbon  dioxide  poisoning.  In  a  few  days  there  is  increased 
fever  with  rapidly  de\'eloping  anaemia,  great  prostration  and  speedy 
enlargement  of  the  regional  lymph-nodes.  Nourishment  is  refused  and 
a  state  of  sonuiolence  supervenes,  interrupted  by  periods  of  excitement. 
The  nose  is  completely  occluded  but  there  is  a  bloody,  ichorous  dis- 
charge. As  a  result  of  the  nasal  plugging,  cyano.sis  comes  on  whenever 
the  infant  tries  to  suckle.  Sometimes  the  membrane  is  \'isible  in  the 
nostrils.  The  extension  of  the  fibrinous  exudate  to  the  pharynx  or 
more  rarely  to  the  oral  cavity  may  occur  in  two  or  three  days  with 
increase  in  the  fever  and  in  the  general  intoxication.  Symptoms  of 
malignant  gangrenous  diphtheria  ma}'  arise,  witli  death  from  the  seventh 
to  the  ninth  day,  frequently  in  an  attack  of  asphyxiation  (Monti). 

Only  about  forty  per  cent,  of  the  cases  recover.  A  favorable  turn 
may  come   after   the   first  or  sometimes  after  the  second  elevation  of 


382  THE   DISEASES   OF    CHILDREN 

temperature,  and  it  is  accompanied  by  a  profuse  purulent  discharge 
containing  particles  of  membrane.  It  is  noteworthy  that  the  first 
stage  may  be  very  mild  and  may  continue  for  several  weeks.  There 
is  an  ordinary  coryza  which  is  suspicious  only  through  being  wholly  or 
mainly  unilateral.  Then  with  a  sudden  onset  of  severe  general 
symptoms,  that  side  presents  the  first  appearance  of  pseudomembrane, 
usually  on  the  septum. 

Microscopic  examination  shows  the  same  typical  appearance  as  in 
pharyngeal  diphtheria.  In  looking  through  many  preparations  only  a 
few  bacilh  are  found,  the  evidence  for  diphtheria  being  the  fibrin-content 
with  the  paucity  of  bacteria.  On  the  other  hand,  that  the  presence  of 
the  diphtheria  bacillus  is  alone  not  sufi^icient  to  make  the  diagnosis  of 
diphtheria  has  been  shown  by  the  researches  of  Trumpp,  Balhn  and 
Schaps,  who  found  them  frequently  present  in  the  nasal  passages  of 
infants  who  were  healthy  or  had  only  simple  catarrhal  processes. 

On  the  same  grounds  many  authors  hesitate  to  regard  a  peculiar 
kind  of  croupous  disease  of  the  nose,  the  memhranous  or  psevdomem- 
branous  rhinitis,  as  a  specific  disease  or  to  rank  it  as  diphtheria.  There 
is  moderate  fever,  with  shght  redness  and  swelling  of  the  nasal  mucosa 
and  a  superficial  fibrinous  exudate.  This  sits  hghtly  on  the  mucosa  and 
can  easily  be  removed,  or  it  may  fall  off  spontaneously,  only  to  be  fol- 
lowed soon  by  a  new  formation,  but  not  causing  any  loss  of  substance  or 
scarring.  There  is  no  tendency  to  involve  the  neighboring  parts,  nor 
are  there  any  symptoms  of  general  toxiemia  either  during  or  after  its 
formation,  and  the  only  sequels  are  local  ones  (Hartmann).  The  only 
thing  pointing  to  diphtheria  is  the  presence — not  without  exception — 
of  diphtheria  bacilh. 

3.   PRIMARY   LARYNGEAL  DIPHTHERIA 

It  is  not  yet  definitely  proven  whether  there  is  a  purely  primary 
laryngeal  diphtheria  or  whether  in  the  cases  in  which  the  disease  appears 
first  in  the  larynx  there  is  not  an  earher  specific  affection  in  some  part 
of  the  pharyngeal  lymphatic  ring  inaccessible  to  inspection. 

The  first  symptoms  are  those  of  a  laryngotracheal  catarrh  with 
moderate  fever.  Then  there  develop  more  or  less  completely  after  a 
few  hours,  or  more  frequently  several  days  and  occasionally  even  after 
a  week  or  two,  the  decided  symptoms  described  on  page  373.  The 
pharynx  and  nose  may  be  perfectly  free  or  show  moderate  inflammatory 
changes,  if  the  process  is  an  ascending  one.  At  the  same  time,  however, 
diphtheria  bacilh  are  found  easily  not  only  in  the  tracheal  secretion  and 
the  particles  of  membrane  expectorated,  but  also  on  the  nasal  mucosa. 
If  the  diphtheria  remains  Umited  to  the  larynx  it  runs  a  much  more 
favorable  and  shorter  course  than  in  secondary  croup. 


DIPHTHERIA  383 

4.  CONJUNCTIVAL  DIPHTHERIA 

Conjunctival  diphtheria  is  a  very  rare  disease,  usually  secondary  to 
a  nasopharyngeal  diphtheria  advancing  through  the  lachrymal  canaleJ. 
Occasionally  it  is  primary  and  then  it  often  sets  up  secondarily  a  diph- 
theria of  the  nose  and  throat.  Impetigo,  eczema  and  cachexia  increase 
the  predisposition  to  it  (Marfan).  According  to  the  chief  local  symptoms, 
two  main  forms  are  recognized,  the  croupous  and  the  diphtheritic.  A 
sharp  distinction  is  not  possible,  for  the  two  forms  merge  into  each  other. 

The  disease  always  begins  on  the  palpebral  conjunctiva  with  red- 
ness and  swelling  and  in  both  forms  it  may  spread  to  the  bulbar  con- 
junctiva, and  also  in  the  severest  forms  to  the  cornea. 

In  croupous  conjunctivitis,  bluish  or  yellowish  white  deposits  are 
found,  sometimes  tliin,  sometimes  tliick,  rich  in  fibrin  but  containing 
few  cells.  When  this  is  removed  the  underlying  mucous  membrane  is 
seen  to  be  red,  roughened  or  like  velvet  and  bleeding  easily.  The  secre- 
tion is  profuse  and  purulent  and  contains  flocculi.  The  bulbar  conjunc- 
tiva is  chemotic,  often  covered  with  haemorrhages  in  the  form  of  dots 
or  streaks,  and  at  times  it  is  partly  covered  with  membrane.  The 
cornea  is  clear  but  may  rarely  show  a  superficial  clouding  with  a  bluish 
film.  As  a  rule  all  these  appearances  develop  in  a  few  days.  The  de- 
posits disappear  in  from  three  to  ten  days,  leaving  a  catarrhal  and 
purulent  conjunctivitis  which  lasts  for  several  weeks.  The  cornea 
remains  intact,  hardly  ever  becoming  permanently  cloudy. 

In  the  diphtheritic  form  the  lids  are  very  red  and  swollen,  often  -uith 
a  board-like  infiltration.  On  attempting  to  separate  them  a  scanty  and 
later  profuse  secretion  flows  out,  a  dirty,  turbid  and  blood-stained  serum. 
In  the  average  form  the  grayish  yellow  membranes,  spotted  mth  blood 
or  brownish  discoloration,  are  scattered  over  the  palpebral  conjunctiva 
to  which  they  are  firmly  attached.  In  the  severest  confluent  form  the 
conjunctiva  from  the  edge  of  the  lids  to  the  palpebral  folds  is  covered  in 
its  whole  extent  with  a  fat-like  membrane,  like  yellowish  gray  rubber. 
Only  a  few  of  the  deposits  can  be  torn  off  and  tliis  causes  decided  bleed- 
ing with  deep  loss  of  tissue.  The  chemotic  pale  yellow  bulbar  cornea,  at 
times  shows  diphtheritic  infiltration  and  is  raised  around  the  cornea  like 
a  wall.  The  neighboring  lymph-nodes  are  swollen  and  hard.  There 
are  usually   more  or  less  general  constitutional  symptoms  with  fever. 

After  three  to  five  days,  or  in  the  confluent  form  eight  days,  the 
secretion  becomes  purulent,  the  so-called  blennorrhcciform  stage.  The 
swelling  and  board-like  infiltration  of  the  lids  subside  and  granulation 
tissue  appears,  followed  by  healing  with  scarring.  The  fate  of  the  cornea 
depends  on  how  soon  the  blennorrhoeiform  stage  develops.  If  it  becomes 
affected  before  this  stage,  it  may  be  destroyed  in  twenty-four  hours, 
either  by  loss  of  epithelium  at  the  centre  with  infiltration  and  a  step- 


384  THE    DISEASES   OF   CHILDREN 

like  loss  of  substances,  or  by  a  shutting  off  of  the  corneal  blood  supply 
(by  pressure  from  the  exudate)  followed  by  a  degeneration  of  the  cor- 
neal tissue  from  the  edge.  In  all  the  severe  cases  the  eye  is  greatly  in- 
jured, amounting  to  complete  blindness  in  some  cases  as  a  result  of  scars, 
staphyloma  or  shrinking  of  the  eyeball  from  a  secondary  suppurative 
iridochorioiditis. 

In  both  of  the  forms  general  symptoms  of  toxa-mia  may  supervene 
on  the  local  changes.  Postdiphtheritic  paralyses  arc  not  rare  after  the 
diphtheritic  form.  In  very  weak  children  even  conjunctival  diphtheria 
alone  may  cause  death  by  a  general  toxa>mia. 

5.  DIPHTHERIA  OF  THE  VULVA 

In  this  extremely  rare,  usually  secondary  localization  of  diphtheria, 
the  mons  veneris,  the  inner  folds  of  the  groin  and  the  labia  majora  are 
swollen  and  red  and  the  regional  lymph-nodes  are  greatly  infiltrated. 
On  the  labia  are  seen  many  scattered  and  confluent  ulcers,  deep  and 
varying  in  size  from  that  of  a  lentil  to  that  of  a  bean,  covered  with  gray- 
ish white,  firmly  seated  masses.  Sometimes  the  whole  vulva  is  covered 
with  a  single  homogeneous  dirty  gray  membrane  under  which  deep  ne- 
crosis is  found.  The  process  sometimes  involves  the  neighboring  organs. 

Diphtheria  of  the  vulva  is  always  attended  by  symptoms  of  marked 
specific  intoxication,  and  it  often  opens  the  jiortal  for  secondary  infec- 
tions. 

In  a  similar  way  the  sexual  organs  in  boys,  with  the  surrounding 
parts  may  be  the  seat  of  diphtheria,  but  this  is  very  rare. 

6.  DIPHTHERIA  OF  THE  SKIN  AND  OF  WOUNDS 

In  diphtheria  of  the  nose,  conjunctiva,  or  car  it  sometimes  happens 
that  the  irritating  discharge  excoriates  the  neighboring  skin  with  the 
formation  of  true  diphtheritic  membrane.  This  is  also  found  exception- 
ally on  the  sides  of  a  tracheotomy  wound.  In  a  similar  way  the  virus 
may  be  carried  to  more  remote  parts  if,  for  any  reason,  they  become 
denuded  of  their  epithelium  by  scratches,  vaccination,  impetigo,  eczema, 
erythema  multiforme  or  other  skin  diseases.  The  affected  parts  of  the 
skin  show  a  doughy  swelling  and  are  covered  usually  with  a  thin,  firmly 
seated  membrane  which  may,  however,  change  by  extensive  inflamma- 
tion and  necrosis  of  the  skin  to  a  thick  deposit  of  a  dirty  grayish  yellow 
or  green  color.  From  the  affected  parts  a  turbid  serosanguinolent  dis- 
charge issues,  often  of  foul  odor. 

Primary  cutaneous  diphtheria  and  diphtheria  of  the  unbroken  skin 
are  very  rare.  In  the  latter  case  there  appear  on  the  skin  red  spots, 
rather  painful,  of  round  or  irregular  outline  and  of  varying  sizes.  In  the 
centre  of  the  spot  a  whitish  yellow  blister  appears  which  soon  becomes 
aggravated.     Immediately  after  this  an  ulcer  forms  which  is  covered 


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a.  Diplitheria  of  a  large  bronchus.  The  pseudomembrane  lies  deep  in  the  mucous  membrane  tissue.  The 
submucosa  is  markedly  infiltrated,  the  mucous  glands  distended. 

6.  Interstitial  myocarditis  in  diphtheria.  The  interstitial  connective  tissue  is  increased  aud  contains  fibro- 
blasts and  round  cells.     The  ai-dema  of  the  interstitial  tissue  and  separation  of  muscle-fibres  can  be  plainly  seen. 

c,  Parench>-matous  degeneration  of  the  kidneys  in  diplitheria.  The  epitheUimi  of  the  tubuli  uriniferi 
are  swollen,  loosened,  and  fallen  off.    The  lumen  is  filled  with  cell  detritus. 


DIPHTHERIA  385 

with  diphtheritic  membrane  (Seitz).  In  many  epidemics  diphtheria  has 
been  observed  on  the  toes  and  fingers  (Colimani,  quoted  by  Filatow). 
As  bacterial  toxins  may  be  absorbed  by  the  skin,  primary  cutaneous 
diphtheria  may  therefore  be  accompanied  and  followed  by  symptoms 
of  toxa-mia;  indeed,  Marfan  makes  the  noteworthy  observation  that 
postdiphtheritic  paralyses  occur  more  frcquentlj'  after  the  cutaneous 
than  after  other  localizations  of  diphtheria,  always  appearing  first  in 
that  part  of  the  body  on  which  the  diphtheria  was  seated.  Even  with 
antitoxin  fatal  results  may  occur  (E.  Adler),  being  usually  caused  by  a 
secondary  infection  with  the  development  of  sepsis. 

7.  DIPHTHERIA  OF  THE  STOMACH  AND  INTESTINE 

This  is  a  very  rare  localization  of  diphtheria,  always  secondary. 
The  oesophagus  remains  free  but  the  stomach  is  not  rarely  attacked  and 
considerable  membrane  is  formed  at  the  cardia,  where  there  is  a  transi- 
tion from  the  squamous  epithelium  of  the  pharynx  to  the  cylindrical 
epithelium  of  the  stomach.  True  diphtheria  of  the  intestine  was  first 
observed  here  by  Duerck. 

The  affection  may  be  unnoticed  during  life.  In  other  cases  there  is 
constant  discomfort,  pain  and  tenderness  in  the  epigastrium  with  un- 
controllable vomiting  of  a  bloody,  foul-smelling  fluid,  and  a  rapid  loss 
of  strength. 

EXANTHEMS 

In  the  course  of  diphtheria,  but  almost  never  before  the  third  day 
of  the  disease,  exanthems  may  appear,  the  frequency  varying  with  the 
character  of  the  epidemic. 

These  eruptions  may  be  like  scarlet  fever,  measles,  rotheln  or  urti- 
caria; they  are  transient  and  much  like  a  serum-exanthem,  but  they  are 
not  identical  with  it,  because  they  appear  also  in  cases  not  treated  by 
antitoxin.  They  remain  a  few  hours,  or  at  the  most  a  day  or  two.  The 
scarlatiniform  exanthem  is  distinguished  from  true  scarlet  fever  by  the 
later  appearance  of  the  rash,  the  absence  of  the  strawberry  tongue  and 
of  the  subsequent  lamellar  desquamation;  the  mottleil  exanthem  from 
measles  by  the  absence  of  the  characteristic  catarrh  and  of  Koplik 
spots;  the  rotheln-like  eruption  from  rotheln  liy  the  much  more  severe 
general  character  of  the  sickness. 

ALBUMINURIA 

Albuminuria  is  present  on  tlie  average  in  about  fifty  per  cent,  of 
all  cases  of  dii)litheria,  sometimes  more  frequently,  sometimes  less  so. 
depending  on  the  character  of  the  epidemic.  It  is  present  not  only  in 
severe  but  also  not  rarelv  in  mild  cases;  and  it  seldom  appears  before 
the  third  day  of  the  disease.    The  amount  of  albumin  varies  greatly  and 

II— 2.5 


386  THE   DISEASES   OF   CHILDREN 

is  not  always  dependent  on  the  severity  of  the  attack,  and  even  in  the 
same  case,  it  may  vary  from  day  to  day.  In  mild  cases  only  traces  are 
present  usually;  in  severe  cases  the  precipitate  is  abundant.  An  amount 
over  two  per  mille  is  seen  only  in  the  severest  diphtheria. 

The  amount  of  urine  is  diminished  but  not  to  the  same  extent  as  in 
scarlet  fever.  Concentration  and  acidity  are  increased  while  the' urea 
output  early  in  the  attack  rises  greatly.  Urobilinuria  and  indicanuria 
are  almost  constantly  present  (Labbe).  The  diazo  reaction  is  present 
in  the  severest  cases  only,  and  these  show  quite  regularly  the  features 
of  an  acute  parenchymatous  nephritis;  great  diminution  of  the  urine, 
even  to  200  c.c.  daily,  proportionately  high  concentration,  a  large 
albumin-content,  hyalin  and  granular  casts,  epithelium,  leucocytes  and 
more  rarely  red  blood  cells. 

The  diphtheritic  nephritis  continues  usually  about  ten  or  fifteen 
days  (the  albuminuria  in  mild  cases  often  for  only  one  to  three  days) 
but  in  protracted  cases  it  may  last  until  death.  It  is  rarely  accom- 
panied by  cedema  and  general  dropsj';  still  more  rarely  by  uraemia,  and 
it  almost  never  becomes  chronic. 

DIPHTHERITIC  HEART  FAILURE 

The  fatalities  in  diphtheria  are  always  more  or  less  dependent  on 
toxic  changes  in  the  heart,  some  cases  being  pure  instances  of  heart 
failure,  such  an  event  occurring  either  in  the  acute  stage  or  in  conva- 
lescence, suddenly  and  unexpectedly,  or  slowly  with  typical  signs.  The 
ultimate  cause  is  still  the  subject  of  much  controversy.  Apparently 
well  grounded  and  hard  to  refute  is  the  theory  of  Eppinger,  of  a  separa- 
tion of  the  fibrils  of  the  heart  muscle  from  their  sheath, — a  myolysis 
from  toxic  anlcma. 

Because  of  the  reduced  strength  of  the  heart,  thrombosis  and  con- 
secutive embolism  may  occur,  immediately  preceding  and  hastening 
death. 

Heart  failure  in  the  acute  stage  is  seen  only  in  the  severest  form  of 
diphtheria.  It  may  occur  wthout  any  warning  or  there  may  be  pre- 
monitory symptoms  of  faihng  circulation  in  progressive  weakness  of 
the  pulse,  coldness  of  the  extremities,  cadaveric  pallor  \\'ith  a  cyanotic 
tint,  swelUng  of  the  liver  and  dilatation  of  the  heart. 

Death  in  convalescence  may  hkewise  be  sudden  and  unexpected  or 
be  heralded  by  the  typical  symptoms.  It  may  occur  not  only  after 
attacks  of  severe  diphtheria  apparently  running  a  favorable  course 
but  also  occasionally  in  relatively  mild  attacks.  The  patients  may  feel 
well  and  have  good  appetites,  looking  bright,  improving  in  color  and 
gaining  strength.  Then  following  on  some  slight  bodily  exertion,  hke 
getting  out  of  bed,  ha^dng  a  movement  of  the  bowels,  or  eating  a  hearty 
meal,  this  catastrophe  occurs.     With  great  pallor  and  involuntary  dis- 


DIPHTHERIA  387 

charge  of  stools  and  urine,  the  patients  sink  back  quietly,  but  some- 
times with  several  cries  and  complaints  of  pain  referred  to  the  abdomen 
(emboUsm  of  the  abdominal  aorta,  Marfan). 

In  other  cases,  especially  in  older  children,  the  heart  failure  occurs 
with  prodromes  of  greater  or  less  duration:  the  pulse  becomes  small, 
compressible,  irregular,  rapid,  v^ith  periods  of  marked  slowness  espe- 
cially shortly  before  death.  The  area  of  cardiac  dulness  is  increased  to 
the  right,  and  at  times  to  the  left,  the  apex-beat  is  weak  and  the  sounds 
are  feeble,  the  first  being  impure  or  even  rough  and  blowing;  toward 
the  end  there  is  gallop-rhythm.  Profound  anjemia,  complete  anore.xia 
and  apathy  deepening  to  somnolence  set  in,  with  unconsciousness 
before  death.  As  in  the  acute  heart  failure,  the  end  may  be  ushered 
in  with  restlessness  and  attacks  of  pain,  and  sometimes  with  unilateral 
paralyses  the  results  of  embohsm  (of  the  abdominal  aorta,  of  the  cerebral 
arteries,  or  of  those  of  the  extremities). 

The  period  of  danger  may  in  rare  cases  last  for  a  long  time  without 
these  grave  comphcations,  or  it  may  speedily  subside.  The  improvement, 
however,  is  frequently  not  substantial  nor  lasting  and  the  patients  later 
succumb  to  an  insiu-mountable  weakness,  the  diphtheritic  marasmus. 

When  the  termination  is  recovery  the  albumin  first  disappears 
(Unruh),  and  later,  the  heart  weakness  and  rapidity  of  the  pulse.  The 
disappearance  of  the  albumin  in  such  cases  may  then  be  considered  a 
favorable  sign. 

DIPHTHERITIC  PARALYSES 

Paresis  and  paralysis  occupy  the  first  place  among  the  nervous 
complications  and  sequels  of  diphtheria.  They  may  appear  early,  or 
late  in  the  form  of  the  so-called  postdiphtheritic  paralyses.  The  paral- 
ysis appearing  early  is  only  locahzed  in  the  pharynx,  occurring  in  the 
very  severe  cases,  from  the  third  to  the  fifth  day.  The  postdiphtheritic 
paralysis  appears  first  in  convalescence  from  one  to  three  weeks  after 
the  disappearance  of  the  membrane. 

It  is  difficult  to  determine  the  frequency  of  postdiphtheritic  paral- 
ysis for  it  is  subject  to  great  fluctuations  according  to  the  type  of  the 
epidemic.  The  average  according  to  Sanne  is  11  per  cent.,  accorthng  to 
Cadet  de  Gassicourt  13  per  cent.,  while  Seitz  fixes  it  at  only  5  per  cent.; 
if  only  cases  that  recover  are  considered  it  is  probablv  from  20  per  cent, 
to  23  per  cent.  With  serum  therapy,  it  appears  to  be  a  Uttle  less  fre- 
quent; at  least,  when  used  promptly,  there  are  fewer  severe  and  multiple 
paralyses.  It  most  frequently  appears  after  descending  diphtheria  and 
likewise  in  the  course  of  malignant  diphtheria;  mild  cases  are  rarely 
followed  bj-  it  and  then  only  with  a  locaHzation  in  the  pharynx. 

It  is  a  flaccid,  usually  incomplete  paralysis  with  partial  reactions 
of  degeneration. 


388  THE   DISEASES   OF   CHILDREN 

There  may  also  be  other  nervous  disturbances,  as  parspsthesia, 
anaesthesia,  rarely  hyperesthesia,  neuralgias,  various  foi'ins  of  cramps. 

The  affection  almost  always  begins  with  a  paralysis  of  the  soft  pal- 
ate and  pharynx,  even  in  those  cases  in  wliich  the  pharynx  was  not  af- 
fected. An  exception  is  seen  sometimes,  but  only  in  niahgnant  diph- 
theria. Paralysis  of  the  eyes  may  follow  that  of  the  pharynx,  then  the 
lower  extremities  are  affected  followed  by  involvement  of  the  upper 
limbs,  the  trunk  and  the  neck. 

The  paralyzed  palate  hangs  in  a  flaccid  condition  and  is  not 
moved  in  speaking  or  swallowing.  As  a  result  of  the  failure  to  close  the 
nasopharyngeal  space  there  are  nasal  voice,  dysphagia  with  regurgita- 
tion of  fluids  through  the  nostrils,  sometimes  aspiration  of  food  with 
attacks  of  couglung  whenever  the  attempt  is  made  to  swallow.  In  addi- 
tion, there  is  lessened  expectoration,  wliich  adds  danger  to  any  affec- 
tion of  the  bronclii  or  lungs. 

The  isolated  palatopharyngeal  paralysis  subsides  in  ten  to  twenty 
days,  occasionally  earlier,  or  it  may  last  a  month.  It  may  be  followed  in 
about  eight  days  by  other  paralyses  (in  about  15  per  cent,  of  the  cases). 

Strabismus  comes  as  a  result  of  the  paralysis  of  the  external  ocu- 
lar nuiscles,  while  the  affection  of  the  cihary  muscle  leads  to  disturbances 
of  accomodation  with  fatigue  on  reading  and  blurring  of  near  objects. 
The  pupil  of  the  affected  side  reacts  only  to  hght.  In  rare  cases  the 
retina  is  involved  and  amblyopia  occurs  or  even  amaurosis. 

Through  paresis  of  the  legs  the  gait  becomes  uncertain  and 
ataxic.  The  weakness  may  be  so  great  that  walking  is  impossible.  The 
patellar  tendon  reflexes  are  lessened.  The  arms  are  not  often  affected 
but  in  rare  cases  there  are  weakness  and  trembhng  of  the  hands  with 
incapabihty  of  performing  dehcate  movements. 

In  the  severest  cases  there  occurs  a  flaccid  paralysis  with  diminution 
of  electrical  irritability  and  absence  of  the  tendon  reflexes. 

It  may  be  impossible  to  hold  up  the  head  because  of  weakness  of 
the  muscles  of  the  neck  and  back  and  the  patients  sit  in  a  bent-over  posi- 
tion or  are  even  unable  to  sit  up.  Paralysis  of  the  facial  muscles  is  very 
rare.  Much  danger  arises  if  the  paralysis  spreads  to  the  respiratory 
aiuscles,  wliile  involvement  of  the  diaphragm  is  sm-ely  fatal.  In  paral- 
ysis of  the  larynx,  wliich  follows  only  after  laryngeal  diphtheria,  aphonia 
and  dysphagia  are  present  with  an  irritating  cough.  Paralysis  of  the 
abductors  of  the  vocal  cords  causes  stenosis,  paralysis  of  the  adductors 
causes  spontaneous  extubation  if  intubation  has  been  done. 

Generalized  paralysis  almost  never  occurs,  multiple  paresis  is  also 
very  rare,  but  their  mortahty  mount.s  to  40  per  cent,  to  50  per  cent., 
while  the  ordinary  forms  show  8  per  cent,  to  10  per  cent.  (Tilatow). 

A  fatal  termination  results  from  paraly.sis  of  the  respiratory  nuis- 
cles,   inspiration-pneumonia,    or   from    exhaustion    in    inanition.     The 


DIPHTHERIA  389 

order  of  the  disappearance  of  the  paralyses,  when  multiple,  is  the  same 
as  that  of  their  development.  The  convalescents  frequently  remain 
weak,  antemic  and  apathetic  for  a  long  time. 

SECONDARY   DIPHTHERIA 

Some  infectious  diseases  seem  to  increase  the  predisposition  to  diph- 
theria: either  through  a  great  weakening  of  the  patient  and  a  lower- 
ing of  whatever  immunity  may  be  present,  or  by  injuring  the  mucosa 
and  thereby  increasing  the  superficial  predisposition.  The  latter  is 
especially  the  case  vdth.  measles  and  the  susceptibiUty  to  diphtheria  is 
increased  so  much  that  the  secondary  diphtheria  always  attacks  those 
mucous  membranes  which  bear  the  brunt  of  the  measles  attack  (croup 
in  measles  ^A'ith  a  toneless  cough  is  always  suspicious  of  diphtheria). 

Diphtheria  is  apt  to  comphcate  scarlet  fever  in  the  second  or  third 
week,  and  curiously  enough  not  in  a  very  severe  form.  On  the  other 
hand  the  type  of  the  disease  is  dangerous  when  scarlet  fever  compH- 
cates  diphtheria,  because  the  diphtheritic  infection  of  the  mucous  mem- 
brane gives  the  streptococci  always  present  in  scarlet  fever  a  chance  to 
cause  septicaemia. 

Not  less  threatening  is  the  combination  of  diphtheria  ■nith  whoop- 
ing-cough, lobar  pneuvionia  or  typhoid  jever  because  the  cliildren  are 
already  of  low  vitaUt}'. 

Tuberculosis  seems   to  increase   the   predisposition   to   diphtheria. 

DIAGNOSIS 

In  cases  which  set  in  with  characteristic  symptoms,  especially  with 
the  decided  formation  of  fibrinous  deposits,  and  which  show  typical 
toxic  symptoms  dm'ing  the  acute  stage  or  during  convalescence,  the 
diagnosis  is  very  easy.  Not  rarely,  however,  neither  local  nor  general 
symptoms  are  marked,  and  yet  it  is  necessary  in  just  such  cases  to  make 
the  diagnosis  quickly  because  the  effect  of  the  specific  treatment  de- 
pends on  its  earliest  possible  use. 

If  the  climcal  features  leave  us  in  doubt,  the  bacteriologic  examina- 
tion will  in  most  cases  give  us  the  desired  aid.  For  this  purpose  a  sterile 
forceps  is  used,  preferably  Loffler's  (Fig.  91),  to  remove  a  piece  of  the 
friable  membrane,  which  may  then  be  transported  in  a  medicine  bottle 
partly  filled  with  water  or  in  a  tightl}'  closed  clean  envelope.  The  mem- 
brane is  subsequently  washed  in  sterile  water  to  remove  the  outer  layer 
of  saprophytes,  and  then  flattened  out  between  two  cover-glasses.  An 
important  characteristic  of  the  membrane  is  shown  by  this  procedure, 
as  to  whether  it  contains  fibrin  or  not.    If  so,  it  does  not  easily  spread  out. 

If  no  membrane  is  present,  a  small  cotton  tampon  held  in  forceps 
or  fastened  on  a  stick  of  wood  is  pressed  lighth^  against  the  affected  part 
of  the  mucous  membrane  and  immediately  rubbed  on  the  cover-glass 


390  THE   DISEASES   OF  CHILDREN 

on  which  a  drop  of  water  has  been  placed.  It  is  then  dried  in  the  air, 
fixed  by  passing  through  the  flame  three  times  and  stained  with  Loffler's 
methylene  blue.  Or  the  cover-glass  before  staining  may  be  mounted 
in  water,  not  in  Canada  balsam,  and  treated  with  a  5  per  cent,  acetic 
acid  solution  in  order  to  differentiate  the  Babes-Ernst  granules  (Cobbet). 

In  diphtheria  much  fibrin  is  seen,  fibrillar  or  plate-like,  as  well  as 
granular  and  flaky,  more  or  less  numerous  cells  and  cell-detritus,  and 
usually  not  very  many  bacteria;  these  are  mainly  or  exclusively  the 
diphtheria  bacilli  w'hich  are  present  in  all  their  forms.  They  lie  in  char- 
acteristic groups  of  two,  three  or  four,  forming  angles  or  parallel  lines, 
or  in  large  groups  massed  in  irregular  confusion  within  the  masses  of 
fibrin. 

The  same  picture  is  seen  in  mixed  infections,  but  with  many  more 
bacteria,  often  more  cocci  and  other  forms  than  diphtheria  bacilli. 

In  a  disintegrating  diphtheritic  false  membrane  are  seen  more  or 
less  structureless  fibrin-masses,  numerous  leucocytes  and  swarms  of 
staphylococci  with  few  diphtheria  bacilli. 

In  nondiphtheritic  deposits  there  is  no  fibrin,  or  only  isolated  threads, 

Fig.  91. 


Loffler's  forceps.     Natural  size.     From  Vierordt's  Diagnosis. 

with  a  great  deal  of  cell-detritus  and  a  great  mass  of  the  most  varied 
forms  of  bacteria,  covering  the  whole  field,  with  perhaps  a  few  bacilli 
suspiciously  like  the  diphtheritic  bacilli. 

The  diagnosis  is  certain  if  nests  of  diphtheria  bacilli  and  fibrin  are 
present;  the  presence  of  fibrin  with  a  scarcity  of  bacteria  makes  it  prob- 
able. If  these  features  are  absent  and  only  a  few  suspicious  looking 
bacilli  are  found,  it  is  doubtful  and  the  only  procedure  to  clear  it  up  is 
the  study  of  a  culture  followed  by  its  confirmation  with  animal  inocu- 
lation. 

By  many,  the  microscopic  examination  is  held  in  little  esteem,  the 
culture  and  ajvinial  experiment  alone  being  considered  of  value.  But  the 
first  method  takes  only  a  few  minutes  for  staining,  it  gives  certain  re- 
sults in  many  cases,  and  it  furnishes  in  the  number  of  diphtheria  bacilli 
and  their  proportion  to  the  other  bacteria  more  valuable  conclusions  in 
mixed  infections  than  an  incompletely  conducted  culture  method.  The 
la'tter  takes  twelve  hours  and  when  carried  out  in  a  remote  laboratory  a 
considerably  longer  time;  the  result  of  animal  inoculation  cannot  be 
known  for  three  days.  In  addition,  the  results  of  the  cultures  are  not 
always  distinct.    It  is  held  by  some  that  animal  inoculation  is  unneces- 


DIPHTHERIA  391 

sary  with  the  use  of  Neisser's  method  of  double  staining  (one  to  five 
seconds  in  acetic  acid-methylene  blue,  2  parts,  alcoholic  cr3-stal-violet 
solution  1  part;  wash,  stain  for  three  seconds  with  chrysoidin  solution; 
wash).  This  method,  however,  is  equally  time-consuming,  for  it  neces- 
sitates growing  the  bacilli  on  blood  serum,  in  an  oven,  for  eighteen  to 
twenty  hours,  and  furthermore  it  is  not  absolutely  trustworthy,  for  in 
the  first  place  many  true  diphtheria  bacilli  do  not  show  the  granules, 
although  Neisser's  requirements  of  growth  have  been  satisfied;  and  in 
the  second  place  Biitschli  has  shown  that  the  granules  arc  no  special 
peculiarity  of  the  diphtheria  bacillus. 

Such  being  the  case,  Ihe  proper  method  to  jollow  is  to  always  use  the 
microscopic  examination;  if  it  gives  dotihtjul  results  a  culture  may  be 
taken,  but  the  administration  of  the  specific  treatment  sho^dd  not  be  de- 
layed for  its  verdict. 

The  bacteriologic  examination  is  to  be  looked  on  as  a  valuable,  but 
with  a  negative  result  not  as  an  exclusive,  diagnostic  means,  and  the 
clinical  features  are  to  be  given  at  least  ec^ual  rank  with  it. 

DIFFERENTIAL   DIAGNOSIS 

1.    RECOGNITION    OF    LOCALIZED     PH.\RYNGEAL    DIPHTHERIA    FROM 
ACUTE    XOXDIPHTHERITIC    AFFECTIONS 

In  all  doubtful  cases  the  indications  of  diphtheria  are:  (1)  the  con- 
temporaneous development  of  a  unilateral,  decided  coryza  with  a  sero- 
sanguinolent  discharge;  (2)  the  contemporaneous  development  of  laryn- 
gitis; (3)  great  swelling  and  hardness  of  the  adjacent  lymph-nodes;  (4) 
the  appearance  of  typical  diphtheria  in  the  family,  in  the  neighborhood 
or  in  the  school  attended  by  the  child. 

Angina  without  Membrane. — Anginas  which  do  not  show  deposits 
by  the  second  day  at  the  latest,  are  hardly  to  be  suspected  of  being 
diphtheria,  even  if  diphtheria  bacilli  are  found  on  the  mucous  membrane. 
Since  diphtheria  bacilli  are  found  in  the  throats  of  healthy  people,  a 
mucous  membrane  ought  not  to  be  considered  as  the  seat  of  diphtheria 
if  it  is  not  altered  in  the  typical  way  by  the  bacilli,  showing  epithelial 
necrosis  and  fibrinous  exudate.  The  occasional  finding  of  large  masses 
of  diphtheria  bacilli  in  a  starting  angina  makes  it  possible  that  a  non- 
specific secondary  infection  precedes  the  diphtheria,  which  will  follow  in 
a  short  time;  or  that  the  bacilli  come  from  a  neighboring  part,  inaccessi- 
ble to  examination  (the  posterior,  under  surface  of  the  tonsils,  the  naso- 
pharynx), but  affected  with  the  diphtheritic  process. 

Failure  to  make  the  diagnosis  at  the  first  examination  is  also  ren- 
dered possible  bj'^  the  scanty  development  at  the  outset  of  the  charac- 
teristic changes  in  the  mucosa,  which  may  be  only  a  delicate,  cloudy  or 
frost-like  deposit.     Further  diagnostic  difficulties  arise  when  different 


392  THE   DISEASES   OF   CHILDREN 

parts  of  the  sanio  region  react  differentl}'  to  tin-  dijihtlieritic  virus,  the 
diphtheritic  exudate  penetrating  deeply  in  places,  but  not  spreading 
ecjually  on  the  surface  and  so  causing  changes  in  the  mucosa  which 
are  seen  in  other  nondiphtheritic  pseudomembranous  affections  of 
the    pharynx. 

Nondiphtheritic  Pharyngeal  Affections  with  Membrane. — These 
are  principally  anginas  or  stomatitis  with  circumscribed  or  diffuse 
catarrhal  purulent  or  pseudomembranous  deposits.  In  all  of  these  a 
secondary  infection  with  diphtheria  bacilli  is  possible  and  must  always 
be  borne  in  mind. 

Follicular  Tonsillitis. — The  swollen  and  suppurating  folUcles  of 
the  tonsils  project  as  small  yellow  dots  above  the  level  of  the  red  mucous 
membrane.  Fever  at  the  onset  is  high,  with  moderate  swelling  of  the 
regional  lymph-nodes,  but  there  is  no  fetor  to  the  breath.  Usually 
there  is  no  more  than  a  family  epidemic.  In  punctate  diphtheria  the 
islands  of  membrane  are  irregularly  formed,  not  glistening,  and  the 
mucous  membrane  is  frequently  only  a  little  or  not  at  all  reddened, 
especially  in  the  beginning  of  the  disease;  the  temperature  is  only 
moderately  elevated. 

Lacunar  Angina. — By  a  deposition  of  catarrhal  secretion  in  the 
lacuna'  there  appear  on  both  inflamed  tonsils  small  spots  of  unequal  size, 
as  in  diphtheritic  lacunar  angina.  The  nondiphtheritic  angina  is  rec- 
ognized: (1)  by  the  color  of  the  deposit,  pure  white,  glistening,  changing 
later  to  a  decided  j^ellow;  (2)  by  the  limitation  of  the  deposits  to  the  ton- 
sils, while  in  diphtheria  by  the  second  or  third  day  the  palate  is  attacked 
and  through  confluence  the  palate  may  be  covered;  (3)  by  the  consis- 
tency of  the  deposit,  which  can  easily  be  rubbed  off  and  pressed  out, 
while  the  fibrinous  diphtheritic  deposits  are  firmly  knit  and  hard  to 
detach. 

Phlegmonous  Tonsillitis  (angina  parenchymatosa).  The  swelling  of 
a  tonsil  is  associated  with  chills,  high  fever  and  general  discomfort.  On 
its  bluish  red  mucosa  there  appears  a  membrane-like,  oval  white  spot, 
sharply  limited  and  made  up  of  tenacious  mucus  and  cast-off  epithelium. 
Secondary  oedema  and  excessive  secretion  of  mucus  interfere  with 
breathing,  while  spasm  of  the  masscters  hinders  chewing.  The  termina- 
tion is  in  abscess-formation,  or  rarely  in  resolution.  Transition  to 
diphtheria  is  possible,  but  in  diphtheria  there  is  never  so  great  a 
degree  of  inflammation  limited  to  one  tonsil,  with  cramp  of  the  muscles 
of  mastication. 

Aphthous  Angina. — It  is  very  rare  to  see  a  primary  locaHzation  of 
aphthous  stomatitis  on  the  isthmus  of  the  fauces.  The  appearance  is 
that  of  small,  yellowish  ulcers,  round  or  oval,  with  slightly  elevated  and 
very  red  walls.  If  these  become  confluent  there  is  a  resemblance  to 
diphtheria,  but  the  distinction  is  made  by  the  presence  of  the  narrow 


DIPHTHERIA  393 

but  pronounced  inflammatory  edge,  the  depression  of  the  plaque,  and 
the  apiiearance  of  disseminated  aphthtc  in  other  parts  of  the  mouth. 

Ulcerative  Tonsillitis  or  Vincent's  Angina. — Ulcerative  stom- 
atitis varies,  even  when  cases  give  the  same  bacteriologic  results.  It 
may  give  a  picture  so  hke  to  that  of  pharyngeal  diphtheria  that  it  is 
often  called  diphtheroid  angina.  In  such  a  case  there  appears  on  one, 
usually  the  right,  tonsil,  a  pseudoniembrane  one  to  two  mm.  thick, 
which  may  spread  to  the  pillars  and  even  to  the  soft  palate,  its  removal 
causing  bleechng  and  loss  of  tissue.  The  flow  of  sahva  is  increased  and 
the  breath  has  a  foul  odor.  There  is  moderate  swelling  of  the  regional 
lymph-glands,  usually  of  the  affected  side,  but  with  no  tendency  to  sup- 
purate. Aside  from  anorexia,  there  is  httle  general  disturbance.  The 
temperature  may  be  slightly  elevated,  or  depressed.  The  throat  becomes 
clear  in  a  few  days  or  the  necrosis  may  be  deep  enough  to  cause  an  ulcer 
filled  with  a  necrotic  mass;  septic  compUcations  maj' result  from  this  or 
diphtheria  may  develop  secondaril3\ 

The  disease  is  distinguished  from  diphtheria  especially  by  the  con- 
sistency of  the  membrane.  It  is  rather  firm  but  not  holding  together  in 
one  piece;  it  consists  of  granular  and  flaky  detritus  and  also  contains 
remains  of  nuclei,  fibrin-threads  and  numerous  bacteria,  especially  the 
Bacillus  fusiformis.  and  spirilla  (Bernheim,  Vincent,  Dopter). 

Herpetic  Angina. — This  is  occasionally  seen  in  children,  coming 
on  with  a  preliminary  period  of  moderately  liigh  fever  for  one  to  three 
days,  with  severe  headache.  Then  herpes  form  on  the  pharyngeal  mu- 
cous membrane  and  break  down  quickly.  After  the  vesicles  Jiave  rup- 
tured there  remain  yellowish  depressions  smTounded  with  an  inflamma- 
tory ring,  and  in  these  a  wliitish  deposit  may  occur.  If  these  run  together 
a  similaritj'  to  diphtheria  may  arise.  The  long  initial  fever,  the  intense 
headache,  the  multiform  appearances  of  the  ulcers  and  the  development 
of  fresh  crops  of  herpes  on  the  mucous  membrane  help  in  differentiating 
the  process. 

The  development  of  herpes  facialis  is  not  distinctive  ;  for  it  is  a  fre- 
quent condition,  in  children,  well  or  sick,  and  may  also  be  present  in 
diphtheria. 

Membrane  after  Tonsillotomy,  Cauterization  or  Caustics. — A 
diphtheria-hke  membrane  is  seen  after  tonsillotomy.  A  mistaken  diag- 
nosis is  possible  only  through  ignorance  of  the  liistory.  Yet  care  must  be 
exercised,  for  secondary  infection  with  chphtheria  is  a  possibihty.  Epi- 
thelial necrosis  and  subsecjuent  ulcer-formation  may  follow  cauterization 
of  the  mucous  membrane  of  the  mouth  or  throat  in  circumscribed  spots. 
The  history  and  the  attendant  circumstances  clear  up  these  cases. 

Affections  of  the  Pharynx  in  Scarlet  Fever. — In  this  disease  the 
picture  may  recall  that  of  lacunar  angina  or  of  punctate  diphtheria, 
or,  as  in  diphtheria,  pseudomembranes  and  a  tendency  to  necrosis  may 


394  THE   DISEASES   OF   CHILDREN 

develop.  In  the  first  case,  before  the  eruption  has  appeared,  the  onset  is 
with  vomiting  (rare  in  diphtheria),  and  high  fever;  and  the  contrast  of 
the  dusky  redness  of  the  pharynx  with  the  snow-wliiteness  of  the  tongue, 
and  later  after  shedding  of  the  coating,  the  characteristic  strawberry 
appearance,  all  point  to  scarlet  fever. 

Diphtheroid  scarlatina,  which  occasionally  does  not  develop  from 
the  simple  angina  until  the  rash  is  fading  differs  from  diphtheria  in  its 
limited  extent — as  a  rule  not  going  beyond  the  tonsils,  and  almost  never 
attacking  the  larynx — in  the  much  greater  tendency  to  tissue-necrosis, 
in  the  very  intense  affection  of  the  glands,  in  the  absence  of  paralysis  and 
in  the  appearance  of  lamellar  desquamation. 

Examination  of  the  urine  may  furnish  important  aids  to  diagnosis. 
The  test  for  the  diazo  reaction  in  diphtheria,  except  in  the  mahgnant 
forms,  is  negative,  while  in  scarlet  fever  it  is  positive  in  40  per  cent,  of 
the  cases.  Urobihnuria  and  indicanuria  are  almost  constant  in  diph- 
theria and  are  rare  in  scarlet  fever  (Labbe). 

Pseudodiphtheria. — Like  ordinary  aphthae  the  symmetrical  ulcers 
on  the  hard  palate,  seen  in  newborn  and  young  infants,  known  as 
Bednar's  aphtha^,  may  under  some  conditions  resemble  diphtheria  very 
strongly.  They  are  caused  by  lesions  of  the  epithelium  in  suckling  and 
swallowing  or  by  rough  cleansing.  In  athreptic  infants  these  small 
circumscribed  lesions  may,  by  the  entrance  of  bacteria,  change  to  sup- 
purating, sharply  limited  ulcers,  which  may  progress  in  a  symmetrica] 
butterfly-fashion  and  cover  nearly  the  whole  of  the  palate.  Membrane- 
formation  may  also  occur  in  places.  In  the  deposit  are  found  detritus, 
pus  cells  and  a  great  mass  of  the  most  varied  forms  of  bacteria,  espe-. 
cially  streptococci  and  staphylococci.  The  temperature  is  normal  or 
shghtly  elevated,  and  the  breath  has  a  cheesy  odor. 

The  ulcers  may  be  the  starting-point  of  a  septic  infection;  on  the 
other  hand  similar  necrosis  of  the  oral  and  pharyngeal  mucous  membrane 
may  appear  in  the  course  of  an  existing  septicemia  (Baginsky) 

While  it  is  easy  to  differentiate  these  ulcers  from  diphtheria  by 
their  characteristic  form  and  the  pultaceous  deposit,  it  is  difficult  to  do 
so  in  the  much  rarer  cases  in  wliich  there  is  the  fibrinous  deposit  on  and 
in  the  mucous  membrane  with  the  formation  of  tough,  elastic  exudates, 
which  are  constantly  renewed  and  wliich  lead  to  necrosis,  the  process 
showing  a  tendency  to  spread  in  the  same  form  to  the  mouth  as  well  as 
to  the  respiratory  and  digestive  tracts  (pseudodiphtheritic  septica?mia, 
Epstein).  Cases  running  such  a  course,  however,  bear  the  plain  evi- 
dences of  septicaemia  in  themselves  and  may  be  recognized  as  such  by 
the  chnical  features.  The  resemblance  to  diphtheria  is  even  greater 
when  coexistent  congenital  struma  or  thymus-hyperplasia  causes  more 
or  less  severe  symptoms  of  stenosis  (Brecelj). 

Thrush. — This  is  a  special  form  of  stomatitis  caused  by  the  thrush- 


DIPHTHERIA  395 

fungus  (Moiiilia  Candida).  The  fungus  penetrates  the  epitheUum  and 
sets  up  an  inflammation  of  the  mucous  membrane,  showing  itself  in 
irritation,  swelHng  and  pain.  The  thrush  colonies  are  round  and  usually 
granular,  and  if  their  growth  is  not  checked,  they  may  run  together  and 
cover  the  greater  part  of  the  mucous  membrane  with  a  thick,  dirty- 
white  layer.  If,  as  is  the  exception,  the  thrush  membrane  is  locahzed 
on  the  isthmus  of  the  fauces,  it  may  then  happen  through  the  difficulty 
of  inspecting  this  part  of  an  infant's  throat  that  the  judgment  of  the 
physician  inchnes  to  diphtheria,  especially  if  coryza  and  hoar.seness  ex- 
ist, as  is  frequently  the  case  with  atropic  infants,  with  fever  from  some 
cause  or  other.  But  careful  inspection  of  the  mouth-cavity  will  .soon 
show  characteristic  isolated  thrush-colonies  and,  at  any  rate,  the  micro- 
scopic examination  will  correct  the  error  by  showing  the  presence  of 
mycelium,  gonidia  and  spores. 

Syphilitic  Stomatitis. — In  hereditary  syphilis,  mucous  patches 
may  rarely  occur  in  the  mouth  in  the  form  of  a  whitish  gray,  round 
infiltration,  sharply  limited  and  somewhat  elevated  on  a  more  or  less 
deeply  reddened  base.  Through  considerable  extension  and  locaUza- 
tion  on  the  tonsils  and  palate,  they  may  resemble  diphtheritic  pseudo- 
membranes,  and  so  much  the  more  if  syphilitic  coryza  and  laryngitis  are 
present.  And  yet  the  patches  may  show  plainly  the  presence  of  fissures, 
while  all  the  accompaniments  of  diphtheria  are  absent,  and  other  symp- 
toms of  lues  may  usually  be  elicited. 

2.    DIFFERENTIATION    OF     LARYNGEAL    DIPHTHERIA     FROM    OTHER    AFFEC- 
TIONS   OF    THE    LARYNX    ACCOMPANIED    BY    STENOSIS 

The  diagnosis  of  a  laryngeal  diphtheria  is  very  easy  when  it  occurs 
in  the  course  of  a  pharyngeal  diphtheria,  but  it  may  be  very  difficult  if 
it  develops  after  the  disappearance  of  a  mild,  unobserved  pharyngeal 
diphtheria,  or  if  it  starts  primarily  in  the  larynx,  does  not  mount  to  the 
pharynx  and  if  at  the  time  of  observation  no  loosening  and  expectora- 
tion of  membrane  have  occurred.  The  possibihty  then  exists  of  con- 
founding the  condition  with  a  nondiphthcritic  infection  of  the  naso- 
pharynx, larynx  and  trachea  which  may  arise  from  some  inflammatory 
or  mechanical  cause  and  go  on  to  the  development  of  dyspnoea,  and  even 
to  a  high  degree  of  stenosis  with  attacks  of  asphyxia.  For  diphtheria, 
there  speaks  the  onset  with  a  rapidly  developing  catarrhal  process,  in- 
crease of  the  initial  hoarseness  even  to  complete  aphonia;  steadily  ad- 
vancing stenosis  even  to  the  maximum  with  attacks  of  asphyxia;  after 
the  attack,  incomplete  relief;  continuation  of  the  dyspnoea  with  a  shght 
degree  of  increase. 

In  all  doubtful  cases,  digital  examination,  but  preferably  laryngo- 
scopic  examination  if  possible,  is  to  be  made;  sometimes  the  question 
is  settled  by  bacteriologic  examination  of  the  pharyngeal  mucus.     A 


396  THE   DISEASES   OF   CHILDREN 

negative  result  of  this  is  not  sufficient  to  exclude  diphtheria;  the  secre- 
tion on  the  coughed-up  or  extracted  tube — in  case  intubation  has  been 
necessary  because  of  asphyctic  attacks — is  to  be  examined  (Marfan);  if 
no  diphtheria  bacilli  are  found  in  it,  diphtheria  may  be  excluded  with 
reasonable  certainty. 

(a)  Nondiphtheritic  Affections  of  the  Nasopharyjix 

Pharyngeal  Angina. — Acute  inflammation  and  swelling  of  the 
pharyngeal  tonsil  is  of  frequt'ut  occurrence  in  newborn  and  young  in- 
fants. Nasal  breathing  is  interfered  with  or  absolutely  prevented  and 
a  watery  secretion  flows  down  the  posterior  pharyngeal  wall.  In  sleep 
it  collects  near  the  entrance  to  the  larynx  and  may  cause  temporary 
obstruction  to  respiration.  The  very  short  duration  and  frequent  repe- 
titions of  the  attacks  which  do  not  appear  when  the  child  is  awake  and 
sitting  up,  as  well  as  the  usually  perfectly  clear  voice,  prevent  mistaking 
it  for  diphtheria. 

Retropharyngeal  and  Retrolaryngeal  Abscesses. — Retropharyn- 
geal abscess  is  seen  rather  freciuently  in  infancy.  .It  may  be  a  suppu- 
rative lymph-node  or  a  penetrating  abscess.  The  primary  cause  in  nurs- 
lings may  be  a  lesion  of  the  mucosa  by  an  infected  finger  of  the  nurse. 
The  abscess  lies  ordinarily  at  the  level  of  the  third  or  fourth  cervical 
vertebra;  and  compresses  the  entrance  to  the  larynx.  There  are  dys- 
phagia, regurgitation  of  fluids,  snoring  respiration  and  finally  a  high 
degree  of  dyspnoea;  a  swelling  may  also  be  seen  externally  in  the  cor- 
responding region  of  the  neck.  The  deep,  gurgling  ring  to  the  voice 
(von  B '  kay)  and  digital  examination  separate  this  from  laryngeal 
diphtheria. 

Macroglossia. — Lymphangioma  of  the  tongue,  the  so-called  macro- 
glossia,  as  seen  in  cretinism  and  myxoedema,  may  when  inflamed  cause 
considerable  obstruction  to  respiration. 

(h)   Nondiphtheritic  Diseases  of  the  Larynx 

Malformations. — The  epiglottis  of  the  newborn  is  normally  rather 
deepl}'  grooved.  This  peculiarity  is  often  so  strongly  developed  that 
the  free  edges  become  movable  to  such  a  degree  as  to  narrow  quite  de- 
cidedly the  lumen  of  the  glottis.  A  congenital  stridor  then  develops, 
with  a  gurgling  sound  on  inspiration  and  difficulty  on  breathing  amount- 
ing to  attacks  of  stenosis  when  excited.  The  history  that  the  symptoms 
have  been  present  from  birth  removes  all  doubt  about  the  nondiphther- 
itic nature  of  the  afTection,  which  disappears  toward  the  end  of  the 
second  year  of  Hfe,  with  the  unfolding  of  the  epiglottis. 

In  very  rare  cases  there  is  seen  a  congenital  adhesion  of  the  anterior 
commissure  of  the  vocal  cords.    The  glottis  is  therefore  narrowed  and  so 


DIPHTHERIA  397 

a  siniplo  laiyngitis  may  cause  a  severe  stenosis.     Such  children  have 
from  l)irth  a  iiuifflccl,  hoarse  voice. 

Laryngitis. — The  differential  diagnosis  of  this  concerns  only  the 
first  stage  of  laryngeal  diphtheria.  The  main  symptoms  in  both  cases 
are  cough  and  hoarseness,  but  their  development  shows  characteristic 
differences.  In  laryngitis,  voice  and  cough  are  loud,  in  diphtheria  they 
grow  steadih'  weaker,  almost  to  silence.  In  addition,  diphtheria  is 
accompanied  by  increasing  d3-spncea  and  swelling  of  the  glands. 

False  Croup. — In  this  form  of  laryngitis  there  appear  sometimes 
in  the  night,  sudden  and  unexpected  attacks  of  asphyxia,  as  a  result  of 
most  acute  inflammatory  swelling  of  the  subglottic  region  (accumulated 
mucus  in  sleep,  obstructed  breathing  and  passive  congestion).  The 
sudden  onset,  absence  of  aphonia,  the  relatively  comfortable  condition 
before  and  after  the  attack,  all  argue  against  its  being  diphtheria. 

Inflammation  of  the  Lower  Vocal  Cords. — This  is  a  form  of 
laryngitis  in  which  a  great  amount  of  oedema  may  develop  in  the  sub- 
glottic region.  The  onset  and  course  strongly  resemble  primary  laryn- 
geal diphtheria  so  that  the  distinction  is  only  possible  by  bacteriologic 
examination. 

CEdema  of  the  Larynx. — Injuries  to  the  laryngeal  mucous  mem- 
brane by  foreign  bodies,  burns,  caustics,  as  well  as  intra-  or  perilaryngeal 
inflammatory  and  suppurative  processes  may  easily  cause  an  acute  oedema 
leading  to  stenosis.  The  historj',  inspection  of  the  mouth  and  pharynx 
and  laryngoscopic  examination  usually  render  the  diagnosis  certain. 
Thrush  in  the  Larynx. — In  very  rare  cases  thrush  may  descend  to 
the  larynx  and  trachea,  perhaps  by  aspiration,  and  the  yellowish  brown 
thrush  fungi  may  be  found  in  abundance  (Massei,  quoted  by  Spiegel- 
berg).  The  diagnosis  is  made  by  examination  of  the  masses  removed 
artificially  or  spontaneously. 

Papillomas  of  the  larynx,  the  most  common  tumors  of  child- 
hood, are  congenital,  or  acquired  as  the  result  of  larvngeal  inflamma- 
tion of  long  duration.  They  may,  according  to  their  position,  size  and 
number  bring  about  a  slowly  increasing  stenosis,  or  they  may  only  occa- 
sionally close  the  lumen  of  the  larj'nx  in  a  valve-like  fashion.  In  the 
latter  instance,  if  a  catarrhal  laryngitis  coexists,  the  condition  may  be 
mi-staken  for  an  attack  of  membranous  croup.  Of  importance  in  the 
differential  diagnosis  is  the  congenital  or  at  any  rate  the  longstanding 
hoarseness. 

((■)   Hyperplasia  of  the  Thymus 

A  well-developed  thymus,  extending  deeply  along  the  sternum, 
may  cause  great  stenosis  in  infants,  because  it  presses  directly  on  the 
bifurcation  of  the  trachea,  where  the  tracheal  rings  are  wide  apart  and 
the  membranous  part  is  extensive,  so  that  slight  pressure  suflJices  to  com- 


398  THE   DISEASES   OF   CHILDREN 

press  the  trachea.  Diseases  causing  active  or  passive  congestion  may 
accidentally  aggravate  the  condition  and  through  ignorance  of  the  pre- 
vious history  diphtheria  may  be  suspected.  Examination  with  the 
Rontgen  rays  will  establish  the  diagnosis. 

(d)  Enlargement  of  the  Thyroid  Gland 

Even  in  the  first  weeks  of  life  enlargement  of  the  thyroid  gland  may 
act  like  enlargement  of  the  thymus,  if  the  gland  is  situated  much  deeper 
than  usual.  The  stenotic  rales  vary  in  intensity  according  to  the  position 
of  the  head,  that  is,  according  as  the  contraction  or  relaxation  of  the 
sternohyoid  muscles  press  the  gland  against  the  trachea. 

(e)    Tuberculosis  of  the  Bronchial  Gland 

Masses  of  tuberculous  lymph-nodes  in  the  region  of  the  trachea  and 
of  the  bronclii  may  also  e.xert  pressure  on  the  air-passages  and  cause 
stenosis.  The  symptoms  described  by  Variot,  Guinon  and  Marfan  are 
very  characteristic:  dyspnoea,  sucking  in  of  the  soft  parts,  and  a  loud 
hollow  sound,  especially  on  inspiration,  which  is  nmffled  when  the  cliild 
is  Ijang  down.  The  long  duration  of  the  trouble,  the  weak  but — so  long 
as  a  recurrent  nerve  is  not  compressed — clear  voice,  the  hoarse  but  not 
silent  cough,  finally  the  absence  of  false  membranes,  exclude  diphtheria. 

3.    DIFFERENTIATION    OF    PRIMARY    NASAL    DIPHTHERIA    FROM    RHINITIS 

A  confounding  of  primary  nasal  diphtheria  with  simple  rhinitis  is 
possible  only  in  the  first  stage.  The  presence  of  diphtheria  bacilli  and 
fibrinous  membranes  on  the  mucosa  or  in  tlie  nasal  secretions,  with 
high  fever  and  swelhng  of  the  lymph-nodes,  confirms  the  diagnosis  and 
excludes  simple  rhinitis. 

Ill  every  coryza  which  runs  its  course  ivith  higli  fever,  'prostration,  swel- 
ling of  the  lymph-nodes  and  a  highly  excoriating,  serosangxiinolent  or  profuse 
purulent  discharge,  the  bacteriologic  investigation  is  of  the  highest  importance. 

4.    DIFFERENTIATION    OF    CONJUNCTIVAL    DIPHTHERIA     FROM 
NONDIPHTHEHITIC    AFFECTION'S 

Aside  from  conjunctival  diphtheria,  formation  of  pseudomembranes 
is  seen  in  blennorrhcea,  in  very  severe  eczematous  or  congestive  catarrh, 
in  pemphigus,  in  herpes  iris  of  the  conjunctiva,  and  in  traumatic  con- 
junctivitis following  burning,  scalding  and  chemical  irritations.  The 
other  symptoms  of  diphtheria  and  the  bacilli  are  absent. 

PROGNOSIS 

The  mortality  of  diphtheria  varies  greatly  according  to  the  type 
of  the  epidemic.  In  injected  cases  it  is,  on  an  average,  from  12  per 
cent,   to   16  per  cent.;    in  uninjected  cases,  according  to  the  figures  of 


DIPHTHERIA  S99 

Zucker  in  St\Tia,  about  40  per  cent,  (in  the  preserum  period, 
according  to  Bayeux,  55  per  cent.).  The  number  of  fatalities  in  the 
separate  forms  of  diphtheria  is  very  varied.  The  figures  in  the  Chil- 
dren's CUnic  in  Gratz  (Pfaundler)  in  the  decade  from  1895-1904  gave 
in  1894  injected  cases  of  localized  pharyngeal  diphtheria,  1.2  per  cent.; 
for  descending  diphtheria  (croup),  17.8  per  cent.;  in  mahgnant  diph- 
theria, 37.1  per  cent,  (see  Fig.  80).  Aside  from  the  form  of  the  diph- 
theria the  termination  is  dependent  on  the  age  and  strength  of  the  pa- 
tient, as  well  as  the  care  and  attention  given;  in  brief,  on  the  attendant 
circumstances.  Diphtheria  in  nurslings  is  most  dangerous  because  of 
the  frequency  of  septic  complications,  and  in  children  up  to  five  years 
of  age,  because  of  the  tendency  of  pharyngeal  diphtheria  to  spread  to 
the  respiratory  tract.  The  disease  is  especially  threatening  when  it 
attacks  radically  weak  and  badly  nourished  children  or  those  depre.ssed 
by  other  diseases  (as  tuberculosis),  or  when  it  appears  as  a  comphcation 
of  measles  or  in  the  course  of  scarlet  fever,  pertus.sis  or  influenza.  Every- 
thing depends  on  an  early  diagnosis  and  the  prompt  injection  of  the  anti- 
toxin, hkewise  on  the  strictest  observance  of  all  the  rules  of  hygiene. 
It  is  necessary  to  exercise  caution  in  giving  a  favorable  prognosis  as  to 
the  outcome,  because  even  apparently  mild  cases  may  suddenly  take  a 
turn  for  the  worse.  Extension  of  the  fibrinous  exudate  to  the  larynx 
alwaj's  renders  the  prognosis  dubious  because  there  may  be  a  sudden 
spread  to  the  bronchial  tree  and  death  may  be  almost  unavoidable  in  spite 
of  the  use  of  the  antitoxin.  The  prognosis  is  hopeless  when  signs  of 
mahgnant  diphtheria  appear :  great  and  painful  sweihng  of  the  glands  wth 
periglandular  oedema,  haemorrhages  into  the  skin  and  false  membrane, 
diazo  reaction  in  the  urine,  signs  of  heart  weakness  and  early  parah'sis. 
As  favorable  signs  may  be  considered  a  profuse  flow  of  sahva,  a 
change  of  the  pharyngeal  secretion  from  a  thick  mucus  to  a  thin  fluid; 
also  the  appearance  of  a  hyperleucocytosis  three  to  four  hours  after  the 
injection  of  the  antitoxin.  (The  serum-injection  is  immediately  followed 
by  a  hypoleucocytosis  and  then  three  or  four  hours  later  in  favorable 
cases  by  a  hyperleucocytosis,  which  is  greater  than  that  existing  before 
the  injection  of  antitoxin.  In  fatal  cases  the  hypoleucocytosis  follow- 
ing the  injection  does  not  >ield  to  a  hyperleucocytosis,  a  proof  that  the 
serum  is  not  acting. — L.  G.  Simon). 

PROPHYLAXIS 

For  efficient  warfare  against  the  plague  of  diphtheria  there  are 
two  plans:  (1)  the  destruction  of  the  bacilli;  (2)  the  closing  of  the 
avenues  by  which  the  bacilh  travel. 

Of  the  highest  importance  is  an  accurate  thagnosis  and  a  knowledge 
of  the  possibihties  of  infection.  Very  few  physicians  have  been  suffi- 
ciently   schooled   in    bacteriologic    methods    or    possess   the    necessary 


400  THE   DISEASES   OF   CHILDREN 

apparatus  for  culture  experiments,  so  in  the  great  majority  of  large 
cities  there  have  been  established  central  laboratories  in  which  exam- 
ination is  made  of  the  cultures  sent  by  physicians  from  cases  in  which 
diphtheria  is  suspected.  It  is  greatly  to  be  desired  that  physicians  should 
use  these  facilities  frequently,  that  they  should  form  the  habit  of  inspecting 
the  pharynx  at  every  visit  to  a  sick  child.  With  membrane,  no  matter 
how  small,  and  especially  with  unilateral,  purulent,  chronic  rhinitis,  ac- 
companied by  fever,  they  shoidd  study  or  have  studied  for  them  the  exudate 
or  discharge.  Until  the  report  on  the  results  of  the  investigation  is  re- 
ceived the  physician  should  regard  every  suspicious  case  as  true  diph- 
theria. With  such  care  he  may  be  able  to  prevent  much  trouble,  espe- 
•  cially  at  the  beginning  of  an  epidemic.  The  patient  and  nurse  should  bj^ 
all  means  be  isolated.  Whether  the  antitoxin  should  be  given  immedi- 
ately depends  on  the  special  circumstances.  It  is  better  to  inject  unneces- 
sarily than  too  late!  When  the  diagnosis  is  established,  all  the  usual 
precautionary  measures  customary  in  other  infectious  diseases  are  to 
be  strenuously  enforced,  disinfection,  forbidding  visitors  in  the  sick- 
room, etc.,  and  these  measures  should  be  maintained  until  complete 
disappearance  of  the  local  symptoms,  in  order  to  avoid  a  spread  of  the 
infection.  For  the  same  reason,  the  patient,  if  at  all  feasible,  should 
remain  in  one  room  throughout  the  attack. 

Because  contagion  may  have  occurred  before  the  presence  of  the 
disease  is  established,  all  cliildren  in  the  immediate  surroundings  are  to 
receive  prophylactic  injections  of  antitoxin.  For  tliis  a  dose  of  200  units  is 
sufficient,  but  for  children  with  other  diseases  such  as  measles,  and  for 
children  under  two  years  of  age,  it  is  better  to  inject  from  600  to  1000  units. 

These  measures  nuist  be  particularly  enforced  if  the  parents  of  the 
children  come  in  contact  in  their  occupations  with  many  people,  as  is 
the  case  with  teachers  and  salespeople,  especially  those  connected  with 
the  handhng  of  food  supplies. 

The  physician  may  also  be  the  carrier  of  infection.  With  certain 
precautions  he  may  avoid  recei"vdng  the  infectious  material,  by  the  use 
of  long  rubber  tubes  on  the  stethoscope  (with  .subsequent  disinfection), 
and  by  standing  behind  the  patient  while  inspecting  the  pharynx, 
looking  down  from  above  and  thus  avoiding  the  danger  of  ha^^ng  the 
patient  cough  in  his  face,  and  at  the  same  time  gaining  a  much  better 
and  deeper  view  of  the  pharynx  (Fig.  92). 

The  immediate  return  of  the  convalescent  to  his  family  is  allowed 
some  days  after  the  disappearance  of  the  membrane.  A  separation  until 
the  bacilli  have  disappeared  from  the  throat  is  not  practically  feasible, 
and  moreover  in  -view  of  their  spreading  to  other  mucous  membranes 
where  they  linger  much  longer,  it  is  useless.  The  raising  of  the  quaran- 
tine includes  a  cleansing  bath  for  the  patient  and  disinfection  of  the 
room  with  formaldehyde  vapor. 


DIPHTHERIA 


401 


Not  until  eight  days  after  disappearance  of  the  membrane  in  un- 
compHcated  cases  is  the  child  to  be  allowed  to  return  to  school.  A 
longer  exclusion  from  school  is  superfluous  because  the  intercourse,  as 
it  ordinarily  exists  among  school  children,  is  not  specially  adapted  for 
the  transmission  of  the  disease.    Greater  care  is  necessary  with  children 

F:g.  92. 


Inspection  of  the  pharynx  from  above,  standing  beiiind  the  patient. 

who  return  to  such  institutions  as  day-nurseries,  because  with  these 
children  "dirt-infections"  are  more  frequent  (Escherich,  Feer). 

The  other  children  of  the  family  must  also  be  included  in  the  exclu- 
sion from  attendance  at  school  unless  they  were  promptly  and  completely 
isolated  from  the  sick  one.  with  the  added  protection  of  immunization 
and  provided  that  they  show  no  signs  at  all  suspicious  of  diphtheria. 

11—26 


402  THE   DISEASES   OF   CHILDREN 

Special  mention  may  be  made  of  two  procedures  calculated  to  pre- 
vent the  development  and  spread  of  diphtheria.  Children  can  easily  be 
instructed  to  allow  inspection  of  the  pharynx  peaceably  and  willingly 
and  this  should  be  done  daily  in  times  of  epidemics.  They  may  also  be 
taught  to  gargle,  so  as  to  bathe  the  posterior  pillars  and  pharyngeal 
wall,  and  then  in  times  of  epidemics  this  may  be  done  twice  daily  with  a 
disinfecting  solution,  preferably  with  peroxide  of  hydrogen. 

TREATMENT 

(a)    SPECIFIC    TREATMENT,    SERUM    THERAPY 

The  entrance  of  xhe  diphtheria  toxin  into  the  body  does  not  have 
wholly  harmful  results,  for  it  also  stirs  up  a  reaction  by  which  not  only 
the  circulating  toxins  become  destroyed,  but  also  the  organism  remains 
immune,  protected  for  a  longer  or  shorter  period  of  time  against  the 
harmful  action  of  the  specific  poison. 

The  condition  of  specific  immunity  may  be  produced  experimentally 
in  animals.  If  a  non-fatal  dose  of  diphtheria  toxin  is  injected  into  an 
animal,  that  animal,  after  showing  symptoms  of  the  disease,  becomes 
immune  to  a  much  greater  dose  of  the  toxin.  By  means  of  regulated 
injections  of  steadily  increasing  amounts  of  the  toxin  it  is  possible  finally 
to  produce  an  immunity  to  any  number  of  times  the  former  fatal  dose 
(active  immunity). 

If  the  serum  of  an  animal  so  treated  is  injected  into  another  ani- 
mal, this  second  animal  shows  itself  resistant  to  a  subsequent  introduc- 
tion of  the  toxin  (passive  immunity);  indeed,  the  serum  from  the  first 
animal  shows  not  only  a  protective  action,  but  also  a  healing  one,  so 
that  when  injected  into  an  animal  the  subject  of  diphtheria,  it  brings 
the  disease  to  a  standstill,  modifies  it  and  hastens  recovery.  For  this 
healing  action,  much  greater  amounts  of  the  serum  are  necessary  than 
to  produce  the  protective  action,  and  so  much  the  greater,  the  further 
the  disease  has  advanced. 

On  this  possibility  of  transferring  the  protective  and  healing  action 
of  the  serum  of  an  artificially  immunized  animal  not  only  from  animal 
to  animal  but  from  lower  animals  to  man,  rests  von  Behring's  serum 
therapy  of  diphtheria. 

Inasmuch  as  natural  and  artificially  acquired  immunity  may  be 
transferred  by  means  of  the  blood  and  its  derivatives,  there  must  be  con- 
tained in  the  latter  specific  protective  substances,  antibodies.  Whether 
these  exist  preformed  in  the  body  or  arc  newly  developed  is  a  mooted 
point.  To  explain  the  action  of  the  antitoxin  on  the  toxin  there  have 
been  advanced  three  theories:  a  physicochcniical  theory  (Arrhenius 
•  and  Madsen),  a  ph^'siological  (Ehrlich),  and  a  biological  (Pauli). 

Explanation  of  Natural  and  Artificially  Acquired  Immunity.— Accord- 


DIPHTHERIA  403 

ing  to  Ehrlich  and  von  Behring  that  substance  which  naturally  in  the 
cells  is  greatly  increased  in  amount  by  the  action  of  the  toxin,  becomes 
the  primary  cause  of  healing  when  it  is  given  off  by  the  cells  into  the 
plasma  of  the  blood. 

According  to  Arrhenius  and  Madsen,  the  saturation  of  toxin  and 
antitoxin  is  really  a  dissociation  of  combinations  with  weak  affinity 
(Dieudonne). 

According  to  Pauli,  the  toxin  and  antitoxin  have  colloidal  charac- 
teristics and  the  very  varied  reactions  of  immunity  are  changes  of  the 
colloidal  condition,  a  more  or  less  complete  neutralization  of  colloidal 
solutions  (W.  Pauli). 

The  antitoxic  serum  is  mainly  derived  from  horses  which  have  been 
highly  immunized  to  diphtheria.  The  value  of  the  serum  is  found  by  its 
action  toward  a  solution  of  the  diphtheria  toxin  of  known  strength. 
That  amount  of  serum  capable  of  neutralizing  one  hundred  times  the 
fatal  dose  for  a  guinea-pig  is  called  an  antitoxin  unit.  If  this  activity 
is  contained  in  one  cubic  centimetre  of  serum,  that  serum  is  called  one- 
fold serum,  but  if  it  is  contained  in  the  hundredth  part  of  a  cubic  centi- 
metre, the  serum  is  called  100-fold.  At  the  present  time,  serum  of  a 
strength  400-  and  500-fold  is  in  the  market. 

In  America,  serums  of  greater  concentration  than  those  mentioned 
are  to  be  found  in  the  market.  Natural  serums  of  700-800-fold  are 
obtainable  as  are  also  equally  strong  serums  which  have  been  concen- 
trated by  chemical  means.  Gibson  has  worked  out  a  process  by  which 
the  serum  globulins,  with  which  the  antitoxic  principle  is  identified,  are 
separated  from  the  serum  albumins  and  the  other  globulins.  These 
antitoxic  globulins  are  soluble  in  an  amount  of  ph3'siologi,cal  salt  solu- 
tion from  one  half  to  one  third  the  volume  of  the  serum  from  which 
they  are  derived.  In  this  way  serums  can  be  concentrated  from  two  to 
three  fold.  Moreover  it  has  been  shown  by  Park  that  by  the  use  of 
this  concentrated  and  purified  antitoxic  globulin  solution  only  about 
one  half  the  number  of  cases  of  the  "serum  sickness"  result  and  its 
severity  is  much  diminished. 

Without  regard  to  the  age  of  the  patient,  the  dose  should  be  1000 
units  for  localized  pharyngeal  diphtheria;  with  the  appearance  of  toxa'mia 
and  in  progressive  diphtheria,  1500  units;  in  laryngeal  stenosis  and  ma- 
lignant diphtheria,  2000  to  3000  units.  If  there  is  no  improvement  after 
twenty-four  hours,  the  injection  should  be  repeated,  perhaps  in  larger 
doses. 

[In  America  physicians  who  have  had  considerable  experience  with 
diphtheria  advocate  the  use  of  much  larger  amounts,  recommending  an 
initial  dose  of  4000  units  for  moderately  severe  pharyngeal  or  nasal 
diphtheria,  if  seen  early;  when  laryngeal  stenosis  exists  or  if  the  toxaemia 
is  decidedly  evident  early  in  the  disease,  at  least  6000  units  should  be 


404  THE    DISEASES    OF   CHILDREN 

given;  if  not  seen  before  the  third  day  8000  or  10,000  units  should  be 
given  in  as  concentrated  a  form  as  possible;  in  progressive  or  toxemic 
cases  another  dose  of  at  least  4000  units  should  be  given  in  six  hours 
and  repeated  at  that  interval  subsequently  until  improvement  is  ob- 
served.    Many  cases  apparently  hopeless  may  thus  be  saved. — A.  H.] 

The  injection  maj-  be  made  with  any  sterilized  syringe  holding  five 
cubic  centimetres.  The  most  suitable  sites  are  those  parts  of  the  skin 
where  the  connective  tissue  is  loose,  like  the  side  of  the  chest  or  the  ab- 
dominal wall.  The  location  should  be  cleansed  in  the  usual  way,  a  fold 
of  skin  raised  and  the  needle  introduced  parallel  to  it  far  enough  so  that 
the  point  is  freely  movable  in  the  subcutaneous  connective  tissue. 
Before  drawing  out  the  needle  a  small  piece  of  adhesive  plaster  is 
placed  over  the  site  of  injection  to  prevent  the  escape  of  serum  and  the 
entrance  of  infection.  Massage  of  the  swelling  raised  by  the  injection  is 
superfluous.  Verj'  often  the  area  around  the  puixfture  is  tender  for 
twenty-four  hours. 

The  serum  hastens  the  melting  away  of  the  pseudomembrane  and 
prevents  a  further  spread  of  the  local  process.  It  also  neutralizes  more 
or  less  completely  the  diphtheria  toxin  which  subsequentlj'  passes  into 
the  circulation  from  the  affected  mucous  membrane.  Clinically  this 
action  is  noticeable  in  twenty  to  twenty-four  hours.  The  picture  re- 
sembles that  of  an  accelerated  natural  recovery.  The  intoxication  does 
not  progress,  the  general  well-being  is  improved,  the  fever  comes  down 
by  lysis  or  crisis,  the  blood  pressure  rises,  and  the  nervous  symptoms 
disappear.  Locally,  the  deposits  are  at  first  cleaner,  glistening  and  then 
more  prominent  as  if  the}'  were  raised  a  little  from  their  base,  sharply 
demarcated  and  surrounded  by  a  more  or  less  well  defined  inflammatory 
area.  On  the  second  day  they  look  softer  and  are  reduced  about  one- 
half.  On  the  third  day  they  have  wholly  disappeared,  or  perhaps  only 
a  small  particle  remains.  If  there  is  a  relapse  and  the  injection  is 
repeated,  the  action  is  similar  to  that  in  the  first  attack  (K.  Zucker). 

Recovery. — An  effect  of  the  antitoxin  is  seen  in  all  cases  wliich  live 
for  at  least  twenty-fom-  hours  after  the  injection,  and  this  effect  is  espe- 
cially noticeable  in  the  changes  in  the  pseudomembrane-.  The  effect  of 
the  serum  and  recovery  are  not  of  the  same  significance  (Wieland),  for 
the  serum  has  no  regenerative  action  on  the  tissue-cells  attacked  and 
destroyed  by  the  toxin  before  the  injection.  Recovery  is  intimately 
dependent  on  the  amount  and  intensity  of  the  absorbed  toxins,  on  the 
point  of  time  at  which  they  enter  the  body,  and  on  the  time  of.  injection 
and  the  amount  of  the  antitoxin.  If  a  dose  of  antitoxin  proportionate 
to  the  severity  of  the  case  is  injected  sufficiently  early,  recovery  may  be 
expected  with  considerable  certainty  under  certain  conditions.  These 
are:  (1)  that  the  cases  are  of  mild  or  average  toxicity;  in  such  cases  the 
action  of  the  toxin  develops  so  slowly  that  the  diagnosis  and  specific 


DIPHTHERIA  405 

therapj^  are  not  too  late.  In  severe  toxic  cases,  on  the  other  hand,  the 
toxin  may  be  formed  in  such  quantities  and  of  such  activity  and  in  so 
short  a  time  passing  into  the  circulation,  and  the  individual  suscepti- 
bility may  therefore  be  so  greatly  increased,  that  injection  of  the  anti- 
toxin even  on  the  first  day  of  the  disease  may  not  be  able  to  prevent  a 
fatal  intoxication;  (2)  that  the  patient  is  not  already  weakened  by  some 
other  disease,  for  in  such  cases  it  needs  only  a  small  amount  of  the  toxin, 
absorbed  before  the  injection  of  the  antitoxin,  to  cause  death;  (3)  that 
no  septic  comphcations  are  present,  for  the  action  of  the  specific  remedy 
is  only  against  the  specific  (diphtheria)  poison,  but  not  against  other  bac- 
terial poisons,  as  it  has  not  the  power  to  combat  any  other  kind  of  bac- 
teria. In  such  cases  therefore  only  partial  success  is  to  be  expected,  to 
the  extent  in  which  diphtheria  toxins  are  taking  part  in  the  di.sease. 

Fig.  93. 


lajectioii  of  serum  m  the  lateral  client-wall. 

Because  the  serum  exerts  no  regenerative  nor  bactericidal  action, 
two  facts  stand  out,  which  are  advanced  by  the  opponents  of  serum 
therapy  as  proof  of  its  uselessness:  (1)  it  sometimes  happens  that  the 
pseudomembrane  spreads  for  twenty-four  hours  after  the  injection, 
even  involving  intact  mucous  membrane.  In  spite  of  the  antitoxin 
there  may  also  develop  albuminuria,  heart-weakness  and  postdiphther- 
itic paralysis;  these  are  symptoms  which  the  judicious  could  not  impute 
to  the  antitoxin,  but  which  are  to  be  credited  to  the  general  intoxica- 
tion existing  before  the  injection.  (2)  No  action  is  observed  on  the  diph- 
theria bacilli  which  remain  much  more  active  and  virulent  and  are 
often  found  for  months  after  the  injection  on  the  mucous  membrane  in 
which  restitution  has  occurred  (in  one  case  after  eighty-two  days. 
Trumpp).  It  is  easy  to  understand  this,  for  the  serum,  derived  by  the 
use  of  the  toxin,  is  able  to  call  forth  only  a  (transitory)  artificial  toxin- 
immunity  and  not,  in  the  strict  sense,  an  infection-immunity. 


406 


THE   DISEASES   OF   CHILDREN 


Following  the  conditions  under  which  the  healing  action  of  the  anti- 
toxin is  possible,  an  almost  certain  success  may  be  expected  in  mildly 
toxic  cases,  the  most  marked  success  being  seen  in  progressive  diph- 
theria with  moderate  toxemia.  While  it  was  customary  in  preantitoxin 
days  to  see  in  such  cases  a  rapid  advance  of  the  fibrinous  exudate  to  the 
bronchial  tree,  now  the  local  process  halts  at  the  bifurcation  of  the 
trachea.  In  progressive  diphtheria  with  great  intoxication,  many  cases 
still  succumb  to  bronchopneumonia  and  rapidly  advancing  heart  failure, 
in  spite  of  the  antitoxin  treatment.  The  success  of  the  antitoxin  in 
malignant  diphtheria  is  much  less;  as  in  the  other  forms  it  depends  on 

Fig.  94. 


Success  of  antitoxin  when  its  use  is  begun  on  the  1st.  2nd,  3rd,  4th,  5th,  and  6th 
day  of  the  disease.  Geh.  Rat.  Lintrner's  Monograph  "Epidemics  and  their  Prevention" 
("  Volkskrankheiten  and  ilire  Bekampfung"), 

the  time  of  injection;  frequently  its  action  is  only  to  prolong  the  dura- 
tion of  the  disease. 

The  value  of  the  antitoxin  treatment  stands  out  clear  and  sharp  in 
statistics.  Even  if  we  except  the  fact  that  the  mortahty  of  diphtheria 
has  fallen  in  the  last  decade  to  one-third,  which  may  rightly  be  in  part* 
attributed  to  a  mildness  in  the  type  of  the  disease,  there  nevertheless 
remain  the  significant  results  of  the  antitoxin  treatment  in  progressive 
and  malignant  cases.  While  two-thirds  of  the  croup  cases  died  in  pre- 
antitoxin days,  now  two-thirds  recover.  Of  the  mahgnant  cases  formerly 
80  per  cent,  to  90  per  cent,  died,  now  about  40  per  cent,  to  50  per  cent, 
(see  Fig.  80,  p.  357). 

*In  the  last  decade,  at  least  in  Styria,  the  mortality  of  noninjected  cases  has  remained 
near  the  usual,  39.5  per  cent.,  as  opposed  to  12  per  cent,  to  13  per  cent,  of  injected  cases 
(Zueker). 


DIPHTHERIA  407 

In  such  a  state  of  affairs  it  is  the  dtily  of  the  physician  to  use  the 
antitoxin  in  treating  diphtheria.  It  is  furthermore  his  duty  to  give  tlie 
injection  at  the  earliest  moment,  because  of  the  fac^  that  recovery  is  so 
much  surer  the  earlier  the  antitoxin  is  given  (see  Fig.  94);  further, 
because  of  the  fact  that  in  every  case  of  diphtheria  there  may  .suddenly 
occur  a  life-threatening  extension  to  the  respiratory  tract  or  an  equally 
sudden  development  of  grave  general  toxaemia;  finally,  because  of  the 
fact  that  experience  has  shown  that  mixed  infections  are  usually  sec- 
ondary to  the  diphtheria-infection. 

These  reasons  \\ill  therefore  impel  the  thoughtful  physician  not  to 
wait  in  doubtful  cases  for  the  result  of  the  hacteriologic  examination, 
especially  if  the  patient  is  very  young  or  reduced  by  another  disease, 
such  as  measles  or  tuberculosis. 

In  older  children,  with  membrane  of  slight  extent  and  absence  of 
general  poisoning,  the  injection  of  antitoxin  may  be  deferred  if  there 
are  other  reasons  for  doing  so,  but  only  in  such  cases  as  may  be  inspected 
several  times  a  day. 

Much  smaller  doses  are  needed  for  prophylactic  injections,  because 
it  is  much  easier  to  protect  the  organism  against  the  diphtheria  toxins 
than  to  combat  an  already-existing  intoxication.  200  units  are  suffi- 
cient (see  page  400).  [In  this  country  the  average  dose  for  prophylactic 
injections  is  at  least  500  units.  Doses  of  1000  units  are  frequently  used 
for  this  purpose  and  cases  are  at  times  reported  where  the  disease 
develops  in  a  mild  form  three  days  to  two  weeks  after  the  injection  of 
1500  units.  The  U.  S.  Pharmacopoeia  gives  500  units  as  the  average 
dose  for  prophylactic  purposes.] 

Because  the  passive  immunity  furnished  by  the  injection  lasts 
scarcely  three  weeks,  the  injection  should  be  repeated  if  circumstances 
demand  it.  The  value  of  tliis  regulation  of  safety  has  been  settled 
beyond  peradventure  by  many  thousands  of  cases. 

Serum  Disease. — The  opponents  of  serum  therapy  assert  not  only 
that  the  antitoxin  is  useless  but  that  it  is  to  be  blamed  for  the  more  fre- 
quent occurrence  of  albuminuria,  paralj^sis  and  diphtheritic  marasmus. 
This  charge  is  just  only  in  so  far  as  such  severe  toxic  cases  are  kept 
aUve  by  the  antitoxin  long  enough  to  develop  the  sequels  of  general 
toxaemia,  while  those  patients  would  have  died  in  the  early  days  of  the 
disease  if  the  antitoxin  had  not  been  given.  It  cannot,  however,  be 
denied  that  the  serum  may  still  produce  certain  symptoms  of  disease, 
which  cannot  be  ascribed  to  its  content  in  antitoxin,  nor  to  the  small 
amount  of  preserving  substance  (0.5  per  cent,  phenol,  chloroform,  etc.) 
but  to  the  horse-serum,  as  such.  In  the  human  system  this  acts  Uke  a 
kind  of  foreign  substance,  in  a  toxic  way.  On  this  account  it  is  desira- 
ble to  use  a  serum  of  the  highest  possible  strength,  in  order  to  make  the 
volume  of  the  injection  as  small  as  possible.    This  disease  is  caused  by 


408  THE   DISEASES   OF   CHILDREN 

simple  horse-serum  as  well  as  that  containing  antitoxin.  Its  manifesta- 
tions appear  after  a  symptomless  incubation-period  of  from  seven  to 
fourteen  days  and  li^t  for  a  few  days,  if  only  small  doses  have  been 
used,  as  is  usual.  They  consist  of  urticaria  followed  by  an  eruption 
whicli  may  resemble  measles,  rotheln  or  scarlet  fever,  in  the  course  of 
which  fever  develops,  with  swelhng  of  the  regional  lymph-nodes;  oedema 
corresponding  in  location  to  that  of  nephritis;  at  times,  albuminuria; 
occasionally  violent  joint  pains  of  brief  duration  with  no  objective 
symptoms  in  the  joints;  leukopenia.  This  disease,  which  has  been  studied 
by  von  Pirquet  and  Schick  very  carefully,  depends  on  an  individual  pre- 
disposition and  the  volume  of  the  serum  injected.  If,  after  an  interval 
of  from  sixteen  to  forty-two  days — or  during  the  time  when  the  specific 
products  of  the  reaction  set  up  by  the  first  injection  are  still  in  the  body 
— a  second  injection  is  given,  the  reaction  appears  immediately  and  is 
shown  by  an  intense  cedema  around  the  site  of  injection,  with  fever  and 
a  general  exanthem  rarely  present.  If  the  reinjection  is  given  after  all 
the  reactive  products  have  been  eliminated,  the  system  then  shows 
only  a  certain  susceptibility  to  the  horse-serum,  the  reaction  is  hastened, 
occurring  after  an  incubation  period  of  five  to  seven  days  (without 
symptoms),  and  is  over  in  a  few  hours. 

From  the  appearance  of  a  specific  local  oedema  or  a  typical  has- 
tened reaction,  it  may  be  confidently  asserted  that  an  injection  has  been 
given. 

The  differential  diagnosis  concerns  only  the  exanthem.  For  a 
serum  exanthem  the  points  are:  (1)  the  time  of  the  eruption,  seven  to 
fourteen  days  after  the  injection;  (2)  first  appearance  of  the  efflorescence 
in  the  neighborhood  of  the  site  of  injection;  (3)  enlargement  of  the  re- 
gional lymph-nodes;   (4)  total  absence  of  mucous  membrane  involvement. 

(b)    GENERAL    TREATMENT 

The  general  treatment  consists  especially  of  hygienic  and  dietetic 
measures.  The  patient  of  course  remains  in  bed,  warmly,  but  not  too 
warmly,  covered.  The  comfort  of  rest  in  bed  is  a  necessity  in  this  dis- 
ease which  uses  up  the  strength  so  quickly.  The  air  in  the  sick  room  is 
kept  pure,  best  by  removing  the  upper  sash  from  a  window.  If  the 
weather  is  so  cold  as  to  prevent  this,  then  for  one  or  two  hours  every 
day  the  window  should  be  opened  wide  and  the  room  flushed  out  with 
fresh  air.  If  the  patient  is  walking  around  at  the  onset  of  the  disease, 
that  room  should  be  chosen  which  is  the  most  spacious,  brightest  and 
sunniest,  and  all  furnishings  that  would  catch  dust  and  germs  are  to  be 
removed.  The  room  is  to  be  provided  with  all  the  furniture  necessary 
to  the  care  of  the  sick  one,  in  order  to  limit  the  intercourse  with  the  rest 
of  the  house  as  much  as  possible.  The  air  of  the  room  must  be  kept  not 
only  constantly  fresh  but  moist,  so  as  to  hasten  the  loosening  of  the 


DIPHTHERIA  409 

pscudomcmbrane.  Water  may  be  vaporized  in  any  suitable  way; 
sometimes  large  sheets  may  be  hung  up  and  wet  with  a  warm,  weak  car- 
bolic solution.  Very  good  service  is  derived  from  a  systematic  steam 
treatment  (see  treatment  of  laryngeal  diphtheria). 

The  diet  must  be  bland  and  nutritious,  and  because  of  the  anorexia, 
as  varied  as  possible,  only  small  appetizing  portions  being  placed  before 
the  patient  at  a  time.  If  the  pain  on  swallowing  is  great,  cold  food  and 
liquids  are  preferable.  Two  or  three  times  a  day  cold  milk  foods  with 
not  too  sweet  fruit  juices  or  cold  .stewed  fruits;  between  these,  two  or 
three  times  a  day,  ice-cold,  diluted  and  acidified  milk  (two-thirds  milk, 
one-third  water,  lo  to  20  drops  of  nitrohydrochloric  acid)  which,  when 
anorexia  is  complete,  may  serve,  alternating  at  short  intervals  with 
fresh  fruit  juice,  as  the  sole  nourishment  for  some  time.  If  desire  for 
warm  food  returns,  in  addition  to  milk  and  milk  foods,  cereal  broths 
may  be  given,  w^ell  cooked  spinach,  potato  or  carrot  soup,  and  light 
farinaceous  foods.  If  plain  milk  becomes  distasteful,  it  may  be  mi.xed 
with  malt-cofTee  or  tea.  Water  may  be  given  to  drink,  or  toast  and 
water  with  lemon  juice;  the  addition  of  much  sugar  is  not  advisable. 
If  there  is  fever,  meat  in  any  form,  meat  broths  and  eggs  are  to  be  ex- 
cluded from  the  diet,  but  alcohol,  coffee,  tea,  chocolate  and  cocoa  may 
be  allowed.  Because  the  whole  organism  is  depressed,  digestion  and 
assimilation  are  below  par  and  care  must  be  taken  to  avoid  giving  food 
too  often  or  in  too  large  amounts.  Overloading  the  stomach  as  the 
appetite  returns  in  convalescence  may  be  dangerous. 

Great  care  must  be  given  to  the  skin.  There  should  be  a  full  bath 
daily,  to  very  sick  patients,  in  bed,  while  a  moderately  .sick  one  may 
have  an  indifferent  soap-bath  of  35°  C.  (.95°  F.).  A  refreshing  measure 
is  sponging  off  with  alcohol  or  cologne-water  after  the  bath;  or,  as  the 
child  sits  in  the  bath,  after  the  whole  body  has  been  soaped,  one  part  at 
a  time  may  be  rinsed  off  with  cool  water. 

The  examiiudion  by  the  physician  is  to  be  carrietl  out  with  the 
greatest  quiet,  everj-  unnecessary  disturbance  of  the  patient  being 
avoided,  especially  if  the  heart  is  weak.  Care  must  be  exercised  not  to 
arouse  the  patient  too  often  nor  to  allow  him  to  move  around  too  much. 
For  eating,  drinking,  defecating  and  urinating,  he  should  be  so  placed 
as  to  use  as  little  strength  as  possible. 

The  fever  is  only  to  be  combated  when  it  lasts  too  long  or  is  too 
high.    The  usual  hydrotherapy  is  sufficient. 

The  diet  may  be  used  to  overcome  the  ordinary  constipation  which 
arises,  by  giving  cooked  fruit  freely,  as  well  as  raw,  grated  or  pressed 
fruit.     Enemas  may  be  necessary. 

The  albuminuria  as  a  rule  demands  no  special  treatment. 

If  heart-weakness  is  threatening,  stimulants  are  to  be  used  imme- 
diately: caffeine  sodium  benzoate.  0.01  to  0.1  Gm.  (J  to  2  gr.)  two  or  three 


410  THE    DISEASES   OF   CHILDREN 

times  a  day;  camphor  benzoate,  0.015  to  0.05  Gm.  (|  to  1  gr.)  every  hour- 
or  two;  digalcn  three  times  a  day,  four  to  eight  drops,  finally  camphor 
and  ether  injections.  If  necessary,  oxygen  inhalations  must  be  used. 
Nursing  and  time  must  accomplish  the  rest,  and  the  physician  should 
see  the  patient  two  or  three  times  a  day.  Later  as  a  tonic,  a  cinchona 
preparation. 

The  danger  of  sudden  heart  failure,  even  in  mild  cases,  lasts  as  long 
as  the  patients  are  ansemic  or  the  pulse  is  arrhythmic,  and  rest  in  or  on 
the  bed  should  therefore  be  observed  until  these  symptoms  are  over- 
come. Following  malignant  diphtheria,  all  the  patients  should  stay  in 
betl  for  two  or  three  weeks  after  the  jjharynx  has  cleared.  Later,  they 
should  be  out  a  great  deal  in  the  fresh  air  to  overcome  the  anamiia, 
which  persists  in  a  slight  degree  for  a  long  time.  Iron  and  arsenic  or 
iron  waters  may  be  administered. 

Isolated  paralyses  recover  by  themselves  in  a  few  weeks.  For 
obstinate  multiple  pareses,  massage,  passive  and  active  gymnastics, 
and  faradization  are  to  be  used;  the  French  authors  (Comby)  praise 
the  favorable  action  of  large  repeated  doses  of  antitoxin.  For  laryngeal 
paralysis  strychnine  is  to  be  given  by  mouth  0.001  Gm.  to  0.003  Gm. 
UV  to  2V  gi'-]  once  or  twice  daily,  or  hypodermically,  0.001  Gm.  two  or 
three  times  a  week  (Henoch,  Heubner).  Gavage  may  be  necessary. 
If  paralysis  of  the  diaphragm  comes  on,  the  phrenic  nerve  may  be 
stimulated  by  the  constant  current,  with  the  cathode  between  the 
trachea  and  the  sternomastoid,  the  anode  on  the  nape  (Heubner, 
Escherich),  with  artificial  respiration  and  inhalations  of  oxygen. 

(c)    LOCAL    TREATMENT 

Great  value  is  to  be  attached  to  careful  cleansing  of  the  mouth  and 
teeth,  the  latter  being  cleaned  with  a  mild  disinfectant  three  times  a  day 
after  each  meal,  the  mouth  being  rinsed  freely.  For  the  hourly  gargling, 
0.1  per  cent,  to  0.3  per  cent,  hydrogen  dioxide,  a  weak  phenol  solution 
(one  dessertspoonful  of  a  5  per  cent,  solution  to  a  quarter-litre  of  water), 
or  diluted  odol  (containing  salol),  or  lemon-water.  If  the  children  are 
small  or  somnolent,  the  mouth  should  be  frequently  washed  for  them, 
or  lemon-water  given  for  drinking.  If  the  children  arc  intelligent  and 
willing,  the  throats  may  be  sprayed  once  or  twice  a  day  with  one  of  these 
solutions.  The  earlier  pernicious  practices  of  swabbing,  forcible  detach- 
ment of  the  membrane  and  painting  of  the  pharynx  with  strong  disin- 
fecting solutions  are  to  be  condemned.  They  are  superfluous  when  the 
antitoxin  is  used  and  are  dangerous  in  malignant  cases. 

A  Priessnitz  bandage  may  be  applied  to  the  neck  and  once  or  twice 
a  day  a  bandage  wrung  out  of  warm  oil  to  protect  the  skin. 

Cleansing  the  nasal  cavities  is  necessary  and  important  in  all  cases 
of  diphtheria.    This  may  be  done  with  the  solutions  already  mentioned, 


DIPHTHERIA 


411 


having  them  lukewarm,  and  pouring  them  in  from  a  teaspoon  or  nasal 
douche.  The  head  must  be  so  held  that  the  fluid  will  flow  horizontally 
backwards  and  not  upwards  into  the  accessory  sinuses. 

Injections  or  irrigations  with  force  are  to  be  avoided,  as  infectious 
matter  may  be  carried  into  the  Eustachian  tube. 

Treatment  of  Nasal  Diphtheria. — In  nasal  diphtheria  irrigations 
are  to  be  alternated  with  insufflations  of  menthol,  0.5  Gm.  (S  gr.), 
sodium  soziodate  1.0  to  2.0  Gm.  (15-30  gr.),  powdered  sugar  20.0 
Gm.  (5  dr.).  The  eroded  areas  on  the  nose  and  upper  lip  are  to  be  pro- 
tected with  an  ointment.     If  the  obstruction  of  the  nostrils  is  so  great 

Fig.  95. 


steam-room.  In  the  adjacent  room  there  is  a  copper  boiler,  heated  by  gas  and  discliarging  steam  through 
a  copper  pipe  in  the  wall  into  the  steam-room;  an  automatic  regulator  keeps  the  water  at  a  constant  level. 
Maximum  capacity,  six  children.     Children's  Clinic,  Gratz,  Prof.  Pfaundler. 

that  drinking  is  impossible  one  or  two  drops  of  a  1  per  cent,  cocaine 
solution  may  be  instilled  into  the  nostrils.  For  subsequent  use  a  weaker 
solution  is  to  be  recommended, — cocaine  hydrochlorate,  0.5  Gm.  (S  gr.), 
boric  acid  4.0  Gm.  (1  dr.),  water  200.0  c.c.  (-1  oz.,  2  dr.)  in  order  to 
reduce  the  swelling  of  the  mucous  membrane  quickh'. 

Treatment  of  Diphtheritic  Otitis. — On  account  of  the  constant 
danger  of  the  spread  of  a  nasopharyngeal  diphtheria  to  the  tube  and 
middle  ear,  the  ears  must  be  examined  daily  and  if  redness  of  the  drum- 
membrane  is  found,  a  warm  solution  of  thymol,  0.1:  50.0,  or  phenol- 
glycerin,  1:  10,  should  be  dropped  in  the  canal.     The  latter  acts  more 


412 


THE    DISEASES   OF   CHILDREN 


surely  but  renders  difficult  the  judgment  on  the  inflammation  because 
a  little  clouding  occurs.  Paracentesis  has  the  usual  indications.  If 
there  follows  a  purulent  discharge,  a  1  per  cent,  to  2  per  cent,  solu- 
tion of   hydrogen  dioxide  should  he  dropped  in  hourly. 

FlQ.  96. 


Improvised  steam-room. 

Treatment  of  Conjunctival  Diphtheria. — In  the  stage  of  board- 
like infiltration  there  should  be  copious  irrigation  with  normal  salt- 
solution  or  boric  acid  solution,  with  ointment  to  the  lids,  and  lukewarm 
compresses  (no  ice).  In  the  blennorrhoeic  stage,  the  treatment  is  the 
same  as  for  any  other  purulent  conjunctivitis;  if  the  cornea  is  not  affected, 


DIPHTHERIA  413 

nitrate  of  silver  may  be  used  sparingly,  in  a  1  per  cent,  to  2  per  cent. 
solution,  or  protargol,  5  per  cent,  to  10  per  cent. 

Treatment  of  Cutaneous  and  Vulvar  Diphtheria. — Sublimate  com- 
presses are  to  be  applied  until  the  nienil)rane  has  disappeared,  then 
borated  iodoform  powder. 

Treatment  of  Laryngeal  Diphtheria. — As  soon  as  signs  of  lar- 
yngeal involvement  appear,  steam-inhalations  must  be  begun  at  once. 
With  them,  about  40  per  cent,  of  antitoxin  cases  may  avoid  operation. 
In  many  children's  hospitals  there  is  a  special  steam-room.  Fig.  95 
shows  such  a  one  in  Pfaundler's  Clinic. 

Sometimes  the  children  are  given  the  inhalations  onh'  periodically, 
for  an  hour  at  a  time.  In  private  practice,  Richaud's  plan  may  be  used 
of  hanging  wet  clothes  in  the  room,  or  submerging  glowing  irons  or  hot 
bricks  in  pans  of  water,  or  in  dwelhngs  of  the  poor  pouring  water  on  the 
hearth-plate.  The  best  plan  is  to  use  a  steam  apparatus  as  recommended 
by  Escherich,  F.  Miiller,  Trumpp,  which  projects  the  steam  against  the 
patient's  face.  To  increase  its  effect  sheets  may  be  hung  over  the  bed, 
improvising  a  steam-room  (see  Fig.  96). 

To  favor  the  elimination  by  the  skin,  hot,  moist  compresses  may  be 
placed  around  the  neck,  or  mustard  poultices.  If  stenosis  sets  in,  a  hot 
bath  followed  by  a  sweat-pack  is  to  be  recommended.  During  the  pack 
a  mixture  of  lime-blossom  and  elder  tea  may  be  drunk. 

If,  in  spite  of  tliis  and  the  antitoxin  treatment,  no  improvement  is 
evident,  but  the  stenosis  increases  and  the  children  become  exhausted, 
operation  is  necessary  to  furnish  free  access  of  air  to  the  lungs.  The 
bloodless  procedure  of  O'Dwyer's  endolaryngeal  intubation  may  be 
chosen,  or  the  cutting  operation  of  tracheotomy. 

Intubation  has  so  many  advantages  over  tracheotomy  that  it 
must  be  considered  first.  One  of  the  main  advantages  is  that  it  is  blood- 
less and  permission  to  perform  it  is  always  obtained,  while  tracheotomy 
is  often  forbidden  by  parents  who  dread  the  knife.  In  addition,  intuba- 
tion consumes  no  more  seconds  of  time  than  tracheotomy  does  minutes. 
It  can  be  done  \\ithout  assistance  and  without  good  illumination,  two 
things  necessary  for  the  proper  performance  of  tracheotomy.  There  is 
no  danger  from  bleeding  or  from  wound-infection.  The  duration  of 
treatment  is  considerably  shorter  because  there  is  no  wound  to  heal  after 
removal  of  the  tube.  Its  results  in  hospitals  are  equally  as  good,  about 
6.5  per  cent,  recoveries  (Siegert) ;  while  in  private  practice  they  are  better 
than  tracheotomy  (Trumpp).  Accidents  during  the  operation  (shock, 
heart  failure,  pusliing  down  of  the  membrane)  are  rare  and  only  to  be 
feared  with  clumsy,  prolonged  attempts.  On  the  other  hand,  distur- 
bances of  swallowing,  cougliing  up  of  the  tube  or  plugging,  and  further- 
more, the  development  of  pressure-ulcers  with  their  sequels  furnish 
more  or  less  severe  difficulties.     Disturbances  of  speech  such  as  chronic 


414 


THE   DISEASES   OF   CHILDREN 


hoarseness,  shortness  of  breath,  etc.,  are  on  the  other  hand  more  fre- 
quent after  tracheotomy  than  after  intubation  (Pfaundler,  Trumpp). 
Intubation  is  contraindicated  if  the  conditions  present  are  such 
that  free  passage  of  air  through  the  tube  cannot  be  expected  or  if  a 
favorable  introduction  of  the  tube  is  for  any  reason  impossible.  In  such 
cases  tracheotomj'  must  be  resorted  to  instead,  and  the  trachea  must  be 
opened  above  or  below  the  isthmus  of  the  thyroid.  If  at  all  possible, 
tracheotomy  is  to  be  done  with  a  tube  already  in  the  trachea,  as  it  is 
much  easier  to  find  the  trachea  then  than  when  it  is  empty.  Dangers 
during  the  operation  are  emphysema,  asphyxia  and  bleeding;  subse- 
quently the  same  complications  may  arise  as  in  intubation,  increased 
bj'  the  possibility  of  infection  of  the  wound  and  secondary  haemorrhage, 
but  dysphagia,  coughing  up  and  obstruction  of  the  cannula  are  far  rarer. 


Fig.  97. 


TECHNIC    OF    INTUBATION 

An  intubation  outfit  comprises  six  or  seven  tubes  of  varying  length 
made  of  metal,  hard  rubber  or  elastic  material;  an  instrument  for  in- 
serting the  tube,  one  for  extracting  it  and  a  mouth-gag.     Fig.  97  shows 

an  ebonite  set.  The 
tubes  are  introduced 
through  the  mouth  into 
the  larynx  and  left  there 
until  the  diphtheritic 
inflammation  has  reced- 
ed, usually  about  three 
days.  The  patient  should 
be  ^Tapped  from  the 
neck  to  the  feet  in  a 
blanket,  and  he  may  be 
intubated  wliile  lying  in 
bed  or  held  on  the  lap  of 
an  assistant,  who  holds 
the  child's  legs  firnJy 
between  the  knees,  vnth 
one  hand  steadying  the 
mouth-gag  and  with  the 
other  holding  the  head 
firmly  in  moderate  ex- 
tension (see  Fig.  98). 
The  tube  is  introduced 
along  the  left  index 
finger  as  a  guide,  which  reaches  deep  in  the  pharynx  and  opens  the  en- 
trance of  the  larynx  by  holding  the  epiglottis  up  against  the  root  of  the 
tongue,  so  that  this  is  pushed  up  and  forward.    Points  to   be  observed 


Intubation  set  with  ebonite  tubes. 


DIPHTHERIA 


415 


in  the  operation  are:  (1)  The  inslrument  must  be  introduced  exactly  in  the 
middle  line  in  order  that  it  may  not  catch  in  any  of  the  different  lateral 
folds  of  the  pharyngeal  mucous  membrane.  (2)  As  the  epiglottis  is  passed, 
the  handle  of  the  introductor  must  be  raised  in  order  that  the  tube  does  not 
ghde  into  the  oesophagus  over  the  root  of  the  tongue  which  half  over- 
hangs the  entrance  to   the  larynx.     (3)   The  Handle  is  again  to  be  lowered 

Fig.  98. 


Maimer  of  hul-imt:  the  chilii  iluiim;  mil 


ajter  the  entrance  of  the  tube  into  the  larynx  in  order  to  prevent  iraumatism 
of  the  anterior  wall  of  the  larynx  by  the  end  of  the  tube  (see  Figs. 
99,  100,  101). 

Extubation  is  accomplished  by  means  of  a  thread  tied  to  the  head 
of  the  tube  and  carried  over  to  one  side  of  the  mouth,  or  if  tliis  is  bitten 
through,  the  tube  may  be  drawn  out  by  tlie  cxtubator,  a  special  instru- 
ment for  the  purpose. 


416 


THE    DISEASES   OF   CHILDREN 


TECHNIC    OF    TRACHEOTOMY 

A  tracheotomy  set  contains:  one  scalpel  for  the  skin-incision,  one 
surgical  and  one  anatomical  forceps  for  separating  the  connective  tissue, 
one  grooved  director  for  raising  the  fascia,  two  blunt  hooks  with  several 
teeth  for  holding  apart  the  layers  of  tissue,  two  sharp  tenacula  for  hold- 

FlG.  99. 

.    r1 


Intubation.  Step  I. — The  index  linger  of  the  left 
hand  hold.-;  up  the  epifjlottis  and  .serves  as  a  guide 
for  the  tube;  the  right  hand  is  lowered. 


Intubation.  Step  II. — The  tube  enters 
the  upper  part  of  the  larynx;  the  right 
hand  is  raised. 


Fig.   101. 


iiig  up  the  trachea,  one  sharp-pointed  knife  for  opening  the  trachea, 
one  blunt-pointed  knife  for  enlarging  the  tracheal  opening,  two  or  three 
cannulas  with  movable  shields,  as  suggested  by  Luer  or  Hagedorn,  or 
two  plain  cannulas,  as   suggested  by  Bruns,  artery  forceps,  scissors. 

The  patient  should  be  wrap- 
ped in  a  blanket  (as  for  intuba- 
tion) and  then  laid  on  a  table 
with  the  neck  put  gently  on  the 
stretch,  which  may  be  conven- 
iently accomplished  by  wrap- 
ping a  bottle  or  other  article  in 
a  towel  and  placing  it  under  the 
nape  of  the  neck.  An  assistant 
should  have  charge  of  the 
ana'sthetization — which  is  super- 
fluous \\ith  a  high  degree  of 
carbon  dioxide  poisoning — and 
he  should  also  watch  carefully  to 
prevent  any  lateral  displacement 
of  the  neck.  The  prehminary  steps  of  cleansing  are  the  same  as  for 
every  cutting  operation.  The  incision,  as  ^^^th  all  subsequent  separa- 
tion of  tissues,  should  be  in  the  median  hne;  extending  for  at  least 
five  centimetres,  in  superior  tracheotomy  to  the  thyroid  isthmus,  in 
inferior  tracheotomy   to   the   sternum.      The   subcutaneous   connective 


Intubation.     Step  III. — The  tube  passes  the  glottis, 
the  right  hand  again  being  lowered. 


DIPHTHERIA  417 

tissue  is  to  be  torn  apart  witii  blunt  instruments,  such  as  closed  haemo- 
static forceps,  the  next  step  being,  with  the  help  of  a  grooved  director, 
to  divide  the  superficial  cer^^cal  fascia  and  the  linea  alba  of  the  sterno- 
hj-oid  muscles,  visible  through  it.  The  next  steps  depend  on  whether 
the  tracheotomy  is  high  or  low.  In  high  tracheotomy,  the  deep  cer\ncal 
fascia  lying  directly  under  the  muscles  must  be  separated  by  a  trans- 
verse incision  from  the  lower  edge  of  a  tracheal  cartilage  and  then 
bluntly  dissected  from  the  trachea  and  drawn  downwards  with  the 
thyroid  gland  enclosed  in  it,  thus  lajdng  bare  the  trachea. 

If  the  loic  tracheotomy  is  being  done,  the  separate  layers  of  the  cer- 
vical fascia  are  to  be  divided  longitudinally  on  a  grooved  director  until 
the  thyroid  gland  is  exposed.  After  division  of  the  lowest  layer,  the 
partly  exposed  trachea  is  to  be  drawn  up  by  two  tenacula  and  freed 
from  any  remaining  areolar  tissue.  A  pointed  scalpel  is  now  introduced 
into  the  trachea  until  a  whistling  sound  tells  that  the  lumen  is  opened, 
when  the  incision  is  to  be  enlarged  sufficiently  (1  to  1.5  cm.)  to  admit 
the  cannula.  (In  a  low  tracheotomy  the  opening  is  to  be  placed  as  higli 
as  possible.)  As  soon  as  respiration  is  easy,  the  cannula  is  to  be  intro- 
duced and  held  in  place  b}'  tapes  around  the  neck.  The  wound  should 
be  carefully  dusted  with  iotloform  and  protected  by  lint  or  rubber  pro- 
tective from  the  tracheal  mucus. 

Difficulties  may  arise  during  the  operation  from  a  large  or  adherent 
thyroid  gland,  a  large  thymus,  numerous  distended  veins  and  rarely 
also  from  arterial  anomalies. 

After  forty-eight  hours  the  cannula  should  be  changed  for  a  clean 
one.  To  prevent  collapse  of  the  soft  parts  they  should  be  held  up  with 
tenacula,  and  an  elastic  catheter  (with  lateral  holes)  should  be  intro- 
duced through  the  cannula  into  the  trachea,  to  serve  as  a  guide  for  the 
removal  of  the  old  and  the  introduction  of  a  fresh  one.  After  a  day  or 
two  a  speaking  cannula  may  be  introduced  and  by  closing  the  external 
aperture  a  test  may  be  made  of  the  degree  of  patulousness  of  the  larynx. 
When  the  child  has  slept  quietly  at  night  with  a  closed  speaking  cannula, 
then  it  maj'  be  entirely  dispensed  with  and  the  wound  allowed  to  heal 
under  an  occlusive  dressing. 

Following  a  secondary  tracheotomy  after  a  long  intubation,  it  is 
wise  to  hasten  removal  of  the  cannula  as  much  as  possible,  in  order  that 
the  breathing  in  the  natural  way  with  the  air-pressure  which  this  exerts 
in  the  larynx  may  hinder  the  formation  of  a  stricture  (v.  Ranke). 

When  extreme  peril  exists,  Fischl's  instantaneous  method  may  be 
followed  by  which  after  the  deep  cervical  fascia  is  reached,  the  trachea 
is  drawn  forward  by  two  tenacula,  and  opened  by  one  cut  passing  through 
all  the  soft  parts  including  the  isthmus  of  the  thyroid.  The  cannula, 
held  ready,  is  immediateh'  thrust  into  the  gaping  opening,  only  the 
cannula  ending  in  a  closed  point  being  suitable.     Pressure  controls  the 

11—27 


418  THE    DISEASES    OF    CHILDREN 

bleeding  which  starts  as  respiration  is  established.  Even  quicker  is  the 
procedure  of  L.  G.  Simon  and  Schinzinger  which  consists  of  fixing  tlie 
trachea  against  the  vertebral  column  and  opening  it  with  one  single  inci- 
sion through  skin  and  soft  parts.  The  index  finger  of  the  left  hand  is 
immediately  pressed  into  the  wound  to  check  the  bleeding  while  the 
cannula  is  guided  along  the  nail  as  the  finger  is  withdrawn. 

Less  dangerous  than  this  mode  of  tracheotomy  is  cricotomy,  which, 
however,  has  the  disadvantage  that  it  always  causes  speech-defect,  an 
interference  with  the  formation  of  the  voice. 


MUMPS— EPIDEMIC  PAROTITIS 

BY 

Dr.  E.  MORO,  of  CIratz 

tr-ojslated  by 
Dr.  FRAXK  X.  WALLS,  Chicago,  III. 


This  epidemic  inflammation  of  the  parotid  gland  is,  as  the  name 
indicates,  of  a  contagious  nature.  For  the  most  part  it  attacks  com- 
pletely healthy  individuals.  Thus  we  have  to  deal  with  a  primary, 
idiopathic  parotitis,  as  distinguished  from  those  inflammatory  processes 
of  the  gland  which  occur  in  the  course,  and  as  a  result  of,  other  dis- 
eases of  an  infectious  character,  and  which  may  be  grouped  as  secondary 
or  metastatic  parotitis. 

Epidemic  parotitis  manifests  itself  in  so  striking  a  manner  that 
neither  the  early  period  of  its  first  accurate  description  (Hippocrates) 
nor  the  numerous  appellations  given  to  it  l^y  the  laity  need  excite  any 
surprise  (Mumps,  Ziegenpeter,  Tolpel).  These  popular  designations 
owe  their  origin  to  the  peculiar  appearance  of  the  patient  caused  by  the 
swelling  of  the  face.  The  humerous  concepts  of  these  names  indicates 
also  that  the  laity  has  long  recognized  the  benign  nature  of  the  disease. 

Pathogenesis,  Anatomy. — The  infection  of  the  parotid  most 
likely  starts  from  the  mucous  membrane  of  the  mouth,  the  micro- 
organisms invading  the  gland  through  Steno's  duct,  and  exciting  an 
inflammation. 

According  to  a  limited  number  of  anatomical  observations,  the 
inflammation  is  confined  to  the  interacinous  tissue  while  the  epithelium 
of  the  glandular  canals  remains  normal.  The  periglandular  and  inter- 
acinous cellular  tissue  appears  to  be  infiltrated  by  a  serous  or  serofi- 
brinous exudation.  If  a  mixed  infection  with  pyogenic  bacteria  from 
the  mouth  does  not  complicate  the  specific  process,  suppuration  of  the 
gland  does  not  occur.  However,  when  there  is  extreme  swelling,  a 
pressure  necrosis  may  occur,  here  and  there  sharply  demarcated  from 
the  other  tissue.  But  in  most  cases  the  process  is  entirely  free  from 
local  complications  and  when  the  exudation  is  absorbed,  complete 
restitution  takes  place. 

Local  Symptoms. — The  most  striking  symptom  is  the  swelling  in 
the  region  of  the  parotid  gland,  which  enables  the  physician  to  make  a 
diagnosis  even  at  some  distance  from  the  patient.     The  location  of  the 

419 


420 


THE   DISEASES   OF   CHILDREN 


swelling  at  times  causes  a  striking  displacement  of  the  lobe  of  the  car 
upwardly  antl  laterally,  a  position  which,  to  a  certain  extent,  is  path- 
ognomonic of  parotid  swelling.  This  horizontal  displacement  of  the  lobe 
of  the  ear,  however,  may  be  frecjuently  wanting,  so  that  we  should  not 
attach  to  it  too  much  importanci'.  The  dimensions  of  the  swelling  are 
subject  to  great  fluctuations.  While  at  times  the  swelling  in  the  fossa 
situated  between  the  ramus  of  the  lower  jaw  and  the  mastoid  process  is 
confined  to  the  region  of  the  parotid,  at  other  times  the  swelling  may 
exceed  these  boundaries  and  sjjread  either  upwards  or  downwards. 

Fig.  102.  Thus    it    may    happen 

tiiat  the  swelling  may 
spread  upward  even  to 
tlie  orbit  and  laterally,  in 
a  diffuse  manner,  over  the 
whole  cheek  down  to  the 
submaxillary  region.  In 
such  a  case  the  entire  half 
of  the  face  appears  swollen, 
the  fissure  of  the  eyelid 
narrowed,  antl  the  conjunc- 
tiva inflamed.  In  some 
cases  the  swelling  may  ex- 
tend to  the  neck  and  even 
(kiwn  to  the  clavicle.  In 
jjilateral  parotitis,  the  swell- 
ing of  the  neck  maj'  join 
the  median  line  and  merge, 
the  neck  assuming  the 
shape  of  a  sausage-like 
tumor.  If  tlie  face  is  in- 
volved to  a  slight  degree, 
the  neck  appears  much 
broader  than  the  face. 

The  skin  over  the 
tumefaction  is  tense  and 
shiny;  its  color  is  ordy  in  rare  cases  slightly  reddened.  The  tumor 
itself  feels  doughy  or  tensely  elastic. 

These  swellings  disfigure  more  or  less  the  faces  of  children;  and  it 
is  evident  that  the  higher  degrees  of  the  swelling  cause  various  incon- 
veniences. In  mild  cases  there  is  frequently  no  sensitiveness  to  pressure 
in  the  parotid.  In  more  marked  swellings  there  is  localized  pain,  espe- 
cially when  the  children  open  their  mouths  to  permit  an  inspection  of 
the  throat,  to  take  food  or  to  chew  a  hard  morsel.  Thus  in  many  cases 
children  experience  difficulty  in  eating. 


Mumps  (left  si.li^ 


II  eifilit  -\t 


MUMPS— EPIDEMIC  PAROTITIS 


421 


An  inspection  of  the  mouth  and  throat  in  most  cases  reveals  nor- 
mal conditions.  At  times  there  is  a  simple  stomatitis  and  pharyn- 
gitis, whose  occurrence  is  favored  by  the  lack  of  attention  to  the  mouth, 
which  is  neglected  on  account  of  the  pain  felt  in  opening  the  jaws.  In 
extreme  cases  the  swelling  spreads  deeply  downwards,  overcoming  the 
natural  resistance  of  the  deep-seated  cervical  fascia  and  we  observe  that 
the  pharyngeal  entrance  is  very  much  narrowed  by  the  protrusion  of 
the  lateral  pharyngeal  walls  and  the  tonsils. 

The  diffuse  extension  of  the  swelling  to  the  neck  produces  more- 
over a  certain  stiffness  in  the  posture  of  the  head.  The  pressure  upon 
the  adjacent  ear,  especially  the  cartilaginous  meatus  and  the  Eustachian 
tube,  diminishes  the  delicacy  of  hearing  and  causes  a  pricking  sensation 
in  the  ear,  a  symptom  met 
with  quite  freciuently  at  the 
very  beginning  of  parotitis. 
If  the  tumor  continues  to 
press  for  a  long  time  upon 
the  facial  nerve,  a  transitory 
facial  paresis  may  occur 
(Falkenheim).  The  effects  of 
local  pressure,  in  severe  cases, 
may  extend  even  to  the  larynx 
and  trachea,  the  disturbed  3s 
circulation  of  the  blood  caus- 
ing a  local  oedema  and  so 
leading  indirectly  to  a  pro- 
nounced laryngeal  stenosis. 

As  a  rule,  the  salivary 
secretion,  which,  in  a  definite 
affection  of  the  parotid  would 
receive  a  good  deal  of  atten- 
tion, remains  normal.  Onlj- 
increased  or  diminished  flow 


41 


40 


39 


37 


36 


Temperature  chart  of  a  moderately  severe 
case  of  mumps. 


rarely  do  disturbances  in  the  way  of 
of  saliva  manifest  themselves.  Nor  does 
the  saliva,  chemically,  show  any  qualitative  or  quantitative  alteration. 
The  diastatic  ferment  and  the  amount  of  potassium  sulphocyanide 
correspond  to  normal. 

General  Symptoms. — The  local  symptoms,  which  are  of  an  exclu- 
sively mechanical  nature,  are  accompanied  by  a  series  of  general  phenom- 
ena of  wliich  fever  is  the  most  prominent.  In  contrast  mth  other  infec- 
tious diseases  of  cliildhood,  this  fever  exhibits  a  wholly  irregular  course, 
so  that  contagious  parotitis  has  no  typical  temperature  curve.  Thus  in 
some  cases,  fever  may  be  absent;  in  others  an  elevation  of  temperature 
occurs  before  a  swelling  of  the  parotid  is  seen,  but  in  most  cases  an  ele- 
vation of  temperature  coincides  witli  the  beginning  of  the  swelling  of 


422  THE   DISEASES   OF   CHILDREN 

the  gland,  dropping  at  times  to  normal  after  a  few  days,  like  a  crisis, 
even  before  the  recession  of  the  local  symptoms.  Sometimes  fever 
accompanies  the  disease  and  slowly  diminishes  with  the  subsidence  of 
the  parotid  swelling.  Just  as  irregular  as  its  course  is  the  height  of  the 
fever.  More  frequent  than  a  high  elevation  of  temperature  up  to  40°- 
41°  C.  (104°-106°  F.),  which  naturally  may  be  accompanied  by  apathy, 
somnolence,  and  dehrium,  are  the  low  temperatures  38°-39°  C.  (101°- 
103°  F.).  If  after  a  few  days  the  inflammation  attacks  the  opposite 
parotid,  the  temperature  generally  again  rises.  If,  during  the  convales- 
cent stage  another  increase  of  temperature  occurs,  compUcations  may 
be  suspected  unless  it  indicates  a  relapse,  which,  however,  is  very  rare. 

At  the  height  of  the  affection,  in  severe  cases,  there  is  a  sweUing  of 
the  spleen  and  of  the  regional  lymph-nodes. 

Prodromes. — A  few  days  before  the  appearance  of  the  parotid 
tumor  the  children  become  cross  and  contrary;  they  lose  their  desire 
for  play,  their  appetites  decrease  and  at  times  they  complain  of  headache. 
Very  frequently  these  general  symptoms  are  accompanied  by  gastric 
disturbances.  Nausea  and  vomiting  ensue,  and  diarrhoea  may  occur  at 
the  very  beginning  of  the  disease  and  may  continue  during  its  whole 
course;  in  fact  cases  in  which  diarrhoea  attains  a  considerable  degree  of 
intensity  are  not  rare.  The  intensity  fluctuates  according  to  the  pre- 
vailing character  of  the  epidemic. 

Course. — The  symptoms  usually  preceding  the  parotid  swelling, 
are  groujjcd  as  the  prodromata  of  mumps.  They  are  not  at  all  charac- 
teristic and  apart  from  certain  local  pain  there  is  manifest  a  feeling  of 
tension  in  the  typical  location.  The  prodromata  hardly  ever  last  longer 
than  one  to  three  daj's,  and  may  be  wanting  entirely. 

It  is  only  with  the  occurrence  of  the  parotid  swelling  that  the  dis- 
ease proper  begins.  The  duration  varies,  depending  essentially  upon  the 
intensity  of  the  swelhng.  Thus  in  light  cases  the  disease  lasts  two  to 
three  days;  in  cases  of  moderate  severity  five  to  eight  days.  But  it 
may  at  times  continue  longer,  so  that  the  process  in  severe  cases,  espe- 
cially when  the  second  parotid  is  involved,  may  not  cease  until  weeks 
have  elapsed.  If  no  complications  ensue,  the  process  runs  along  smoothly 
as  a  rule,  leaving  beliind  no  functional  disturbances. 

The  disease  proper  is  preceded  by  an  incubation  stage,  lasting 
eighteen  to  twenty-two  days.  The  very  length  of  this  incubation  i)criod 
is  to  a  certain  extent  typical,  so  that  famihes  with  many  children  some- 
times do  not  get  rid  of  the  mumps  for  half  a  year. 

Contagiousness  and  Disposition. — Parotitis  is  a  peculiarlv  epi- 
demic affection,  as  shown  by  its  spread  in  families,  educational  insti- 
tutions, schools,  in  towns,  cities,  and  provinces.  Almost  without  excep- 
tion the  infection  takes  place  directly,  from  child  to  child,  but  cases 
have  been  reported  in  which  a  direct  transmission  could  be  positively 


MUMPS— EPIDEMIC  PAROTITIS  4:23 

excluded  and  an  indirect  infection  through  third  persons  or  objects 
(even  letters)  must  be  assumed.  Such  cases  would  indicate  that  the 
exciting  agent  of  parotitis  has  a  greater  resisting  power  than  the  conta- 
gion of  the  acute  exanthemata.  Statistics  show  that  the  di.sease  appears 
more  frequently  during  the  cold  than  during  the  warm  season.  There 
exist  no  relations  to  other  infectious  diseases  in  the  sense  of  an  increase 
or  decrease  of  predisposition  to  parotitis  during  the  course  of  or  after 
convalescence  from  other  infectious  diseases. 

With  the  recovery  from  parotitis  the  body  almost  always  acquires 
a  specific  immunity  against  tliis  disease  which,  as  a  rule,  continues 
through  hfe,  but  some  cases  of  genuine  relapses  have  been  observed  and 
reported  (Gerhardt,  Hochsinger,  Scliilling,  Nirmier,  etc.). 

Children  between  the  ages  of  four  to  fifteen  years  have  the  greatest 
disposition  to  infection,  whereas  those  under  two  years  are  rarely  af- 
fected. Primary  parotitis  in  infancy  is  exceedingly  rare.  Falkenheim 
reports  such  a  case  in  an  infant  seven  moftths  old,  and  Wliite  one  in  a 
newborn  child.  We  may,  accordingly,  assume  that  the  infant  possesses 
against  parotitis  a  pecuhar  natural  congenital  immunity  as  he  does 
against  other  infection;  or  accept  Soltmann's  explanation  that  the  in- 
complete development  of  the  parotid  and  the  narrowness  of  its  duct 
ofTer  unfavorable  conditions  for  the  infection. 

The  character  of  the  epidemic  is  of  especial  interest.  It  has  already 
been  stated  that  in  certain  epidemics,  gastro-intestinal  phenomena  are 
conspicuous.  But  its  contagiousness  too,  is  dominated  by  the  "genius 
epidemicus,"  parotitis  in  many  epidemics  being  marked  by  an  uncanny 
infectiousness,  whereas  in  others  the  affection  appears  in  only  isolated 
cases;  so  that  the  brothers  and  sisters  of  an  infected  child  are  spared. 
In  many  epidemics,  regularly  only  one  gland  is  involved,  whereas  in 
others  there  is  a  bilateral  parotitis.  As  in  other  infectious  diseases,  the 
character  of  the  epidemic  varies,  especially  with  regard  to  complications. 

Complications. — The  comphcations  and  sequelae  of  parotitis  are 
as  rare  as  they  are  diverse.  The  best  known,  because  most  peculiar, 
complication  is  that  described  by  Hippocrates,  a  unilateral  orchitis  (or- 
cliitis  parotidea).  This  comphcation  is  observed  beyond  the  age  of 
puberty  more  often  than  in  childhood.  Henoch  never  saw  a  single  case. 
However  such  well  authenticated  cases  have  been  reported  that  there 
can  be  no  doubt  concerning  the  close  relations  of  the  two  organs  in  paro- 
titis. In  the  course  of  certain  epidenucs  orchitis  appears  much  more 
frequently  than  in  others.  At  times,  strange  to  say,  the  testicle  alone  is 
specifically  affected,  while  the  parotid  remains  free.  In  quite  an  analo- 
gous manner,  although  still  more  rarely,  the  genital  tract  of  girls  is  in- 
volved in  the  parotitis  process.  Included  in  these  rarities  are  unilateral 
swelling  of  the  mamma,  of  the  labia  majora,  and  of  the  ovaries.  Perhaps 
we  M'Ould  discover  these  benign  comphcations  more  frequently  if  we 


424  THE    DISEASES   OF   CHILDREN 

paid  particular  attention  to  them.     There  have  been  reported  a  few  cases 
of  sinuiltaneous  swelling  of  the  thyroid,  thymus,  and  lachrymal  glands. 

\'ery  frecjuently  the  submaxillary  gland  is  involved  along  with  the 
parotid,  swelling  so  much  that  it  may  be  felt  as  a  hard  tumor  at  the  angle 
of  the  lower  jaw.  Sometimes  the  submaxillary  gland  is  specifically 
affected  and  the  parotid  is  spared  (so-called  submaxillary  mumps). 

Beyond  the  involvement  of  glands,  complications  on  the  part  of 
other  organs  especially  during  cliildhood  are  interesting  and  noteworthy. 
Foremost  among  these  is  nephritis  (Henoch,  Mettenheimer,  etc.).  The 
period  of  its  appearance  varies.  Most  commonly  it  sets  in  during  the 
stage  of  convalescence,  concomitant  parotitis  and  nephritis  being  very 
rare.  The  nephritis  has  almost  always  a  ha>morrhagic  character  and 
must  be  distinguished  from  those  symptoms  of  renal  irritation  wliich, 
under  the  aspect  of  a  febrile  albuminuria,  not  infrecjuently  manifest 
them.selves  in  the  course  of  ]iarotitis.     Its  course  is  as  a  rule  benign. 

Other  comi)hcations  to  b'e  noted  are  disturbances  of  the  central  ner- 
vous system,  such  as  convulsions,  delirium,  and  severe  psychoses,  at- 
tended sometimes  by  transitory  dementia  and  loss  of  memory  (Heubner). 

In  other  cases,  somatic  disturbances  of  the  nervous  system,  such  as 
rigidity  of  the  pupils,  paralysis  of  the  ocular  muscles,  monoplegia,  and 
sensor}'  disturbances  have  been  observed  after  parotitis.  All  these 
phenomena  point  to  the  existence  of  cerebral  focal  lesions,  the  severest 
form  of  which,  under  the  pictm'e  of  a  post-parotitic  meningo-encephalitis, 
may  result  in  death  (Maximovitch  and  Gallavardin). 

More  freciuent  are  complications  of  the  auditory  organ.  Otitis 
media  may  be  understood  from  the  nature  of  the  parotitis  itself  and 
from  the  proximity  of  the  infection  (Steno's  duct  and  the  Eustachian 
tube).  But  even  without  jireccding  inflammation  of  the  middle  ear 
parotitis  may  be  attended  with  severe  labyrinthine  affections,  a.ssoci- 
ated  with  deafness,  vertigo,  and  intense  headache,  and  as  experience  _ 
teaches,  yield  a  very  unfavorable  prognosis. 

Grancher  and  Longuet  were  the  first  to  report  cases  of  endoperi- 
carditis  after  mumps,  and  subsequently  many  cases  were  reported. 
Finally  may  be  mentioned  the  rare  complications  on  the  part  of  the 
joints.  These  behave  much  like  gonorrhoeal  and  scarlatinous  articular 
affections,  but  as  a  rule  have  a  milder  course  (Lannois  and  Lcmoine). 

Etiology. — The  etiology  of  parotitis  is  as  yet  by  no  means  suffi- 
ciently explained.  True,  the  character  of  the  disease  presupposes  the 
existence  of  a  specific  pathogenic  factor,  but  the  bacteriological  find- 
ings at  hand  are  few,  deficient  and  unsatisfactory.  Deserving  of  great 
appreciation  are  the  investigations  of  Bcin  and  Michaelis  (1897),  accord- 
ing to  which,  in  mumps,  motile  diplostreptococci  were  demonstrated 
in  the  buccal  secretion,  in  pus,  and  once  in  the  blood;  and  F.  Pick  (1902) 
in  cultivating    micro-organisms  from  the  fluid  obtained  by  puncture  of 


MUMPS— EPIDEMIC  PAROTITIS  425 

the  inflamed  parotid,  which  he  identified  as  the  organisms  of  Bein  and 
Michaelis.  On  the  other  hand,  Schottniiiller,  after  puncture  of  the 
gland  under  the  most  careful  precautions,  found  the  secretion  to  be  per- 
fectly sterile.     The  demonstration  of  transmission  failed  in  every  case. 

Diagnosis. — The  diagnosis  is  made  from  the  local  symptoms.  In 
the  differential  diagnosis  there  need  be  considered  only  such  other  glan- 
dular swellings  in  the  region  of  the  ear  and  under  the  angle  of  the  jaw  as 
appear  either  spontaneously  or  associated  witli  inflammatory  processes 
in  the  buccal  cavity  (for  instance,  Pfeiffer's  glandular  fever).  But  if 
we  take  into  consideration  the  typical  seat  of  the  parotid  tumor,  which 
corresponds  exactly  to  the  topographical  situation  of  the  gland,  and  if 
even  with  intense  swelling  a  redness  of  the  skin  is  wanting,  we  may, 
even  before  the  supimration  of  other  Ij'mphatic  tumors,  safely  avoid 
confounding  them  with  mumps  and  vice  versa.  Secondary  and  metas- 
tatic parotitis  are  considered  elsewhere.  Great  diagnostic  difficulty  is 
encountered  only  in  those  cases  in  which  the  submaxillar}'  gland  alone 
is  specifically  affected,  the  diagnosis  here  must  be  based  only  and  exclu- 
sively on  the  course  of  the  disease  and  on  data  in  the  history. 

Prognosis. — In  spite  of  the  number  and  severity  of  comphcating 
contingencies  the  prognosis  is  nevertheless  favorable.  But  for  an  ade- 
quate estimation  of  the  prognosis  we  must  contrast  the  greatly  prepon- 
derating number  of  cases  running  their  course  without  leaving  any  trace 
with  the  rare  occurrence  of  more  serious  complications,  which  nowadays 
are  of  considerable  casuistic  interest. 

Prophylaxis. — The  prophylaxis  is  confined  to  the  isolation  of  the 
sound  children  from  those  already  affected.  However,  in  view  of  the 
benign  character  of  the  affection  and  in  view  of  the  fact  that  paro- 
titis in  childhood  is  more  easily  endured  than  in  advanced  age,  such 
precautionary  measures  are  for  the  most  part  unheeded.  I  believe  that 
it  is  contrary  to  the  general  welfare  to  permit  the  further  spreading  of 
the  affection  by  the  non-observance  of  these  simple  rules,  apart  from 
the  fact  that  with  increasing  age  the  disposition  to  infection  decreases 
considerably.  Isolation  therefore,  as  far  as  practicable,  should  be  recom- 
mended.   The  duration  of  the  contagiousness  is  six  weeks. 

Treatment. — The  treatment  is  local  and  symptomatic.  In  order 
to  relieve  the  tension  of  the  skin,  warm  oils  or  emollient  salves  may  be 
applied  on  a  cotton  dressing  loosely  over  the  swollen  parts.  In  obstinate 
cases,  in  order  to  facilitate  absorption  within  the  inflamed  gland,  the 
affected  portion  should  be  anointed  with  iodide  of  potash  ointment  or 
iodovasogen,  once  or  twice  a  day.  The  mouth  should  be  carefully 
cleansed,  in  order  to  prevent  stomatitis.  Moreover,  rest  in  bed  must  be 
ordered  as  long  as  there  is  fever;  confinement  to  the  room  until  there  is 
no  inflammatory  glandular  swelling,  and,  in  order  to  regulate  the  diges- 
tion and  to  avoid  local  pains,  a  liquid  diet. 


TYPHOID  FEVER— ABDOMINAL  TYPHUS 

BY 

Professor  R.  FISCHL,  of  Prague 

tr.\nslated  by 
Dr.  frank  X.  WALLS,  Chicago,  III. 


By  this  name  we  designate  an  acute  specific  infectious  disease 
primarily  localized  in  the  bo\yel.  whence  the  causative  bacilli  enter  the 
lymphatics  and  the  blood.  (It  is  now  believed  that  the  localization  in 
the  bowel  does  not  take  place  until  after  the  invasion  of  the  blood  cir- 
culation.) It  is  met  with  in  childhood  about  as  frequently  as  at  other 
periods  of  life. 

With  regard  to  the  mode  of  transmission,  it  was  formerly  thought 
to  be  really  contagious,  but  this  of  such  slight  degree  that  nobody 
cared  particularly  to  isolate  the  patients.  Certain  observations,  how- 
ever, communicated  especially  by  Dr.  Robert  Koch  from  liis  careful 
study  of  an  epidemic,  warn  us  to  have  more  regard  for  the  contagious 
factor — not  only  to  disinfect,  and  remove  dejecta,  urine  and  sputa, 
which  for  some  time  have  been  considered  as  the  most  important  sources 
of  the  disease,  but  also  to  isolate  the  patient.  Even  the  early  investi- 
gators warned  against  infection  through  the  water  used  for  drinking, 
culinary  purposes,  or  bathing  (a  sad  example  of  which  Prague  has  for 
j'ears  been  furnishing),  yet  as  Koch  points  out,  this  is  to  be  less  regarded 
than  those  mild  cases  which  clinically  are  hardl)-  noticed;  healthy  indi- 
viduals whose  evacuations  contain  typhoid  bacilli,  and  must  be  appre- 
ciated as  disseminators  of  the  disease.  We  should  not  however  follow 
him  in  this  curt  disregard  for  hitherto  prevailing  views,  although  we  may 
infer  from  his  observations  and  from  his  protective  measures  thus 
successfully  established  that  besides  the  hitherto  combated  sources  of 
infection  there  are  others  that  should  be  considered. 

Where  typhoid  fever  is  endemic  we  observe  often  enough  that  in- 
fants, even  those  that  are  nourished  exclusively  on  their  mothers'  or 
nurses'  breasts,  become  affected  and  infect  their  nurse.  In  such  cases 
the  infection  must  have  taken  place  through  other  than  the  usual  chan- 
nels, and  the  water  used  for  bathing  lias  been  suspected.  Cow's  milk 
too,  may  be  instrumental  in  spreading  the  disease,  infected  by  water 
used  for  the  purpose  of  diluting  the  milk,  or  by  flies  carrying  the  bacilli, 
etc.     Cases  of  intra-uterine  infection,  generally  resulting  in  death  and 

426 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  427 

expulsion  of  the  fa?tus,  have  rather  a  casuistic  interest.  Transmission 
through  suction  is  asserted  b}-  some,  denied  by  others,  and  as  a  rule  is 
difficult  to  prove,  in  that  an  atTected  nurse  can  infect  the  baby  in  many 
ways.  On  the  other  hand,  there  are  cases  in  which  nurses  suffering  from 
typhoid  fever  of  moderate  severity  have  taken  certain  precautions  and 
have  attended  the  infants  during  the  whole  course  of  the  fever  without 
infecting  them, — an  experiment  too  daring  to  be  imitated. 

The  cause  of  the  disease  is  the  typhoid  bacillus,  described  by  Eberth 
and  first  cultivated  by  GafTky, — a  cylindrical  bacillus  with  rounded 
ends,  and  provided  with  a  chaplet  of  cilia,  presents  lively  transverse 
and  longitudinal  movements.  It  easily  takes  the  aniline  dyes  and 
rapidly  gives  them  up  again,  grows  on  the  usual  culture  media,  is  facul- 
tative anaerobic,  and  ceases  to  grow  at  a  temperature  above  46°  C. 
(115°F.).  The  appearance  of  the  cultures  is  not  very  characteristic,  that 
on  potatoes  being  the  most  striking  one,  a  moist,  lustrous,  mucous 
coating,  looking  like  parchment.  A  knowledge  of  the  appearance  of 
bouillon  cultures  is  important,  since  these  show  a  diffuse  turbidity 
within  12  to  24  hours,  but,  with  transmitted  light,  exhibit  darker  stripes, 
resembling  the  vein-like  markings  on  marble.  In  appropriate  culture 
media  the  typhoid  bacillus  does  not  protluce  gas  nor  ferment  sugar,  nor 
cause  indol  formation,  nor  docs  it  coagulate  milk. 

The  great  powers  of  resistance  and  endurance  of  the  bacilli  assumed 
on  the  strength  of  experiments  made  by  Janowski  and  others,  by  virtue 
of  which  they  are  able  to  live  in  the  water  and  in  the  ground  for  a  long 
period  and  even  resist  freezing,  are  controverted  by  Koch  on  the  strength 
of  his  own  investigation.  He  admits,  however,  that  they  will  withstand 
desiccation,  which  does  not  hurt  their  vitalitA^  A  consideration  of  the 
clinical  features  of  the  disease  ami  the  post-mortem  appearances  points 
to  the  production  of  a  soluble  poison  by  the  bacilli,  but  the  production 
of  such  a  poison  has  as  yet  been  impossible. 

Immunization  experiments  and  serotherapeutic  trials  will  be  dis- 
cussed in  the  chapter  on  treatment. 

It  is  important  to  differentiate  the  typhoid  bacillus  from  the  bac- 
terium coli,  which,  morphologically,  culturall}',  and,  as  recent  investi- 
gations made  by  G.  Sallus  show,  genetically,  is  closely  related 
to  the  bacillus  typhosus.  The  bacterium  coli,  according  to  the  same 
investigator,  is  said  to  form  the  same  aggresin  (in  the  sense  of  Bail)  as 
the  typhoid  bacillus.  If  so,  the  identity  of  the  two  species,  as  already 
assumed  by  many,  becomes  ver}^  probable.  Likemse,  reports  from 
various  sources,  made  during  the  last  few  years  concerning  para- 
typhoid bacilli,  show  that  in  this  group  of  schizomycetes  there  exist 
many  similarities  and  affinities,  and  that  the  cultural  differences  are 
for  the  most  part  insufficient  for  a  separation  of  the  species. 

The  cultivation  of  the  bacillus  may  be  made  from  the  hving  or  the 


428  THE   DISEASES   OF   CHILDREN 

dead  subject.  The  demonstration  during  life  has  a  great  prophylactic 
value,  and  we  may  justly  hail  it  as  an  essential  advance  that,  by  the 
method  elaborated  by  Drigalski  and  Conradi,  we  are  able  to  cultivate 
the  typhoid  bacillus  from  the  dejections  during  the  very  first  days  of 
the  disease  and  to  separate  them  from  other  bacteria.  The  former 
methods,  such  as  those  of  Eisner,  Piorkowski,  and  others,  were  inade- 
quate. By  tills  means  it  was  possible  for  Koch  to  recognize  early  and 
isolate  the  cases  during  the  epidemic  at  Gelsenkirchen.  Other  places 
where  the  organisms  may  be  found  in  the  hving  are  the  spleen  (from 
which  the  germs  are  obtained  by  puncture,  a  procedure  that  cannot  be 
recommended)  and  in  a  very  high  percentage  of  cases  the  rose  spots 
where  they  may  be  sought  for  without  danger  to  the  patient. 

In  the  cadaver,  the  surest  places  to  find  the  bacillus  are  the  spleen, 
the  mesenteric  lymph-nodes  and  the  gall  bladder,  where,  according  to 
the  observations  made  at  the  Prague  Pathological  Institute  and  con- 
firmed elsewhere,  bacilli  may  be  almost  always  demonstrated. 

Besides  the  culture  methods  wliich  enable  us  to  differentiate  the 
typhoid  l)acilU  from  morphologically  similar  organisms,  and  wliich  are 
based  essentially  on  the  absence  of  gas  formation,  of  indol  production, 
and  of  coagulation  of  milk,  we  possess  quite  a  reliable  method  of  recog- 
nition in  agglutination  which  will  be  discussed  later. 

Pathological  Findings. — While  the  post-mortem  findings  in  an 
adult  are  quite  characteristic,  those  in  children,  especially  in  the  first 
years  of  Ufe,  are  much  less  typical;  ulceration  for  the  most  part  is  want- 
ing and  the  changes  are  confined  to  a  shght  infiltration  of  the  agminated 
and  later  of  the  solitary  folUcles,  such  as  occurs  in  severe  enteritis. 
Moreover,  we  find  in  the  earlier  stages  catarrhal  swelhng  and  hyper- 
aimia  of  the  mucosa  in  the  lower  part  of  the  ileum  and  in  the  region  of  the 
ileocecal  valve,  at  times  extending  also  to  other  portions  of  the  small 
intestine,  and  considerable  infiltration  of  the  mesenteric  glands  corre- 
sponding to  the  altered  portions  of  the  bowel.  It  is,  however,  the  soft 
and  enlarged  spleen  that  particularly  indicates  typhoid  fever.  Other 
alterations  that  may  be  mentioned  are  a  parenchymatous  degeneration 
of  the  liver  and  kidneys,  muscular  degeneration  of  the  heart,  oedema 
and  hyper semia  of  the  meninges  and  cerebral  substance,  lobular  and 
lobar  pneumonia  are  almost  constantly  present,  hypersemia  of  the  bron- 
chial mucous  membrane,  and  such  secondary  infectious  processes  as 
suppuration  of  the  middle  ear,  gangrene  of  the  cheeks,  suppuration  of 
the  parotid,  purulent  joint  affections,  etc. 

We  see  from  the  above  that  the  typical  necroses,  ulcers,  and  cica- 
trices are  missing,  and,  as  Marfan  forcibly  remarks,  we  frequently  have 
presented,  especially  in  infants,  a  pathologico-anatomic  picture  more 
indicative  of  a  septica?mia  and  a  complete  explanation  only  follows  a 
bacteriologic  examination. 


TYPHOID  FEVER— ABDOMINAL  TYPHUS 


429 


Fig.  104. 


Course  of  the  Disease. — In  childhood  the  course  of  abdominal 
typhoid  is  relatively  mild,  and  the  mortality  correspondingly  small. 
During  the  time  I  have  been  preparing  this  article  and  in  spite  of  the 
great  prevalence  of  typhoid  in  our  city,  I  have  been  unable  to  obtain 
any  material  from  post-mortem  examinations  of  cliildren  to  have 
pictures  made. 

Filatow  states  that  the  mortality  of  children  varies  between  3  and 
10  per  cent,  against  17  to  25  per  cent,  in  adults,  yet  severe  epidemics 
occur,  for  instance  one  reported  by  Guinon  in  Paris  with  a  mortality  of 
17.5  per  cent. 

Moreover,  the  course  of  the  disease  is  shorter  in  cliildren,  the  dura- 
tion of  the  several  stages  being  less  and  symptoms  which  later  are  liighly 
dangerous,  as  intestinal  haemorrhages  and  perforation,  are  exceedingly 
rare,  there  being  no  ulceration,  or  only  rarely  and  this  only  in  older 
children.  The  onset  as  a  rule  is  unnoticed,  showing  itself  in  different 
ways,  disposition  to  sleep  at  an  un- 
usual hour,  restlessness  at  night,  loss 
of  appetite,  mild  disturbances  of 
digestion  such  as  eructations,  mod- 
erate vomiting,  and  constipation; 
thus  inconspicuously,  the  disease  is 
slowly  ushered  in  until  the  fever 
with  its  somewhat  characteristic 
steps  is  present.  The  latter  may 
be  divided  properly  into  three 
stages — a  period  of  gradual  ascent 
of  temperature  (called  stadium  in- 
crementi  by  Filatow),  continuous 
fever,  and  defervescence.  The  first  period  exliibits  an  evening  ex- 
acerbation of  temperature  each  morning  higher  than  the  preceeding 
morning  and  then  a  steady  rise  of  fever.  In  the  second  stages  the 
difference  between  morning  and  evening  temperature  is  only  shght,  .5 
to  1.5°  C.  (1°  to  4°  F.),  and  in  the  tliird  stage  the  temperature  descends 
to  the  normal  in  the  morning  wliile  in  the  evening  there  is  a  shght 
increase,  and  this  gradually  diminishes.  The  aggregate  duration  of  the 
fever  in  light  and  medium  cases  is  2h  to  3  weeks,  of  wliich  3  to  5 
days  may  be  allotted  to  the  first  stage  and  as  many  days  to  the  third 
stage,  while  the  period  of  continuous  fever  lasts  10  to  14  days. 

As  a  matter  of  course,  there  are  numerous  deviations  from  the  type 
just  described.  There  may  be  a  longer  duration  of  the  fever  (up  to  40 
days  and  more),  the  so-called  "formes  prolongees"of  Cadet  de  Gassicourt. 
as  well  as  a  shorter  or  abortive  course;  sudden  onset  with  sharply  rising 
temperature,  observed  especially  in  quite  young  cluldren:  a  critical  fall 
of  the  fever;  the  so-called  inverted  type,  in  which  the   morning  tem- 


Normal  temperature  curve  iu  typhoid  fever. 


430  THE   DISEASES   OF   CHILDREN 

perature  is  liigher  than  the  evening.  For  the  most  part  the  fever  remains 
at  a  mean  height  in  the  first  years  of  Hfe  not  exceeding  39°  to  39.5° 
C.  (102°-103°  F.),  but  occasionally  there  are  considerable  elevations  of 
temperature  up  to  41°-42°  C.  (106°-107.6°  F.),  which  are  usually 
well  borne  by  the  youthful  patients  as  is  fever  generally.  A  sudden 
drop  of  the  temperature,  with  .simultaneous  bad  appearance  of 
the  patient,  wliose  face  becomes  pale  and  pointed,  is  as  a  rule 
indicative  of  intestinal  ha'morrhage  or  perforation  and  is,  therefore,  a 
sign  of  bad  omen. 

The  frequency  of  the  pidse  increases  slowly  and  not  excessively,  so 
that  the  rate  closely  corresponds  to  the  fever  or  is  even  slower.  Only 
in  case  of  the  occurrence  of  some  dangerous  complications,  in  cardiac 
weakness  and  in  the  death  agony  does  the  pulse  become  thready  and 
hardly  perceptible.  Dicrotism  is  frequently  present,  but  on  account  of 
the  smallness  of  the  arterial  tube  it  cannot  be  easily  detected  by  the  pal- 
pating finger.  During  the  period  of  convalescence,  the  pulse  frecjuently 
becomes  slower  and  at  times  irregular. 

Concerning  the  blood  pressure  we  have  investigations  made  by 
Carriere  and  Doncourt,  frojii  which  we  learn  that  at  the  beginning  of 
the  affection  the  arterial  tension  drops  from  13  or  14  to  8  or  7,  but 
during  the  second  phase  'slowly  rises  from  9  to  28.  During  the  period 
of  defervescence  and  convalescence,  comes  a  second  decrease  of  pressure, 
followed  slowly  by  a  return  to  normal  conditions.  Increase  of  blood 
pressure  may  occasion  intestinal  haemorrhages,  pulmonary  congestion, 
delirium,  etc.  Myocarditis  is  not  always  accompanied  by  a  decrease 
of  the  blood  pressure. 

The  younger  the  child,  the  less  the  accompanying  nervous  symptoins, 
which  for  the  most  part  are  confined  to  apathy  and  restlessness  at  night. 
The  typhoid  state  which  is  so  characteristic  in  the  adult  with  highly 
flushed  or  pale  cheeks,  injected  conjunctivte,  dull  expression,  etc.,  is 
rare.  At  most  a  hypcr-cxcitability  prevails,  such  as  tossing  about  in 
bed,  tremor  of  the  hands,  hypcra>mia  of  the  face,  uncanny  lustre  of  the 
eyes  or  finally  even  convulsions.  A  furibund  delirium,  alternating  with 
deep  stupor,  points  to  a  cerebral  disturbance  especially  when  rigidity  of 
the  neck  and  back  muscles,  picking  of  the  bed  clothes,  deep  sighing, 
grinding  of  the  teeth,  and  other  symptoms  characteristic  of  meningitis 
set  in.  During  convalescence  aphasia  may  occur  as  I  have  seen  in  a  case 
observed  jointly  by  Eschcrich  and  me,  which  presented  also  symptoms 
of  idiocy.  Similar  cases  have  been  reported.  In  another  case  under 
my  observation  after  defervescence,  there  occurred  an  eclamptic  attack 
lasting  a  day  and  a  half  with  resulting  imbecility.  Delirium  from  inan- 
ition, melancholic  depression,  transitory  paralysis  of  various  muscles, 
etc.,  are  by  no  means  rare  sequela;  of  grave  typhoid  and  all  of  these  point 
to  a  severe  intoxication. 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  431 

The  loss  of  appetite  (though  usually  not  absolute),  the  high  jever, 
the  diarrhcca,  and  the  insufficient  night's  rest  lead  in  ohildren  to  great 
emaciation,  which  at  times  Ijecomes  extreme,  but  during  convalescence 
conditions  quickly  improve.  Often  the  hair  falls  out  and  is  replaced  by 
a  thin,  lustreless  aftergrowth;  but,  in  contrast  to  the  adult,  rarely  is 
any  permanent  harm  done.  The  finger  nails  exhibit  transverse  furrows 
and  flutings;  often  the  nails  drop  off,  and  new  ones  grow  in,  but  not,  as 
Feer  believes,  such  as  are  characteristic  of  scarlet  fever.  Under  the  trophic 
disturbances  we  note  desquamation  of  the  skin  such  as  described  by 
Hamernik,  in  the  form  of  branlike  or  large  scaly  exfoliations  of  the  trunk 
and  of  the  extremities,  while  the  face,  hands,  and  feet  remain  unaf- 
fected. Rachmaninow  observed  this  desquamation  in  one-third  of  all 
the  cases  of  typhoid  fever  in  children  that  came  under  his  notice.  It 
appeared  either  during  the  stadium  decremcnti  or  not  until  after  the 
temperature  reached  normal  and  continued  from  8  to  14  days.  The 
severity  of  the  disease  had  no  influence  on  its  occurrence. 

Patients  frequently  have  a  peculiar  craving  for  certain  undigestible 
foods,  obstinately  rejecting  what  liquid  food  is  offered  them,  and  their 
aversion  lasts  as  long  as  the  fever.  Frequently  as  early  as  the  period 
of  deferescencc,  and  regularly  during  convalescence,  ravenous  hunger 
is  present  which  demands  firmness  on  the  part  of  the  physician  and 
his  assistants,  since  the  foods  which  are  permitted  do  not  satisfy  the 
appetite  and  more  food  must  be  refused. 

In  the  typhoid  of  childhood,  the  tongue  often  presents  a  character- 
istic appearance.  It  seems  to  be  narrowed,  covered  at  first  with  a  gray 
transparent  coating  and  later  with  a  thick  white  deposit,  sharply  con- 
trasting with  the  dark  red  border  and  the  clean  moist  tip.  There  are, 
however,  as  I  have  seen  repeatedly,  cases  in  which  during  the  whole 
course  of  the  disease  the  tongue  showed  no  coating  or  at  most  only  a 
slight,  breath-like  turbidity.  The  clearing  of  the  tongue  begins  at  the 
tip,  which  gives  rise  to  the  so-called  "typhoid  triangle"  with  its  apex 
towards  the  root  of  the  tongue.  A  dry  tongue,  looking  as  if  it  had  been 
smoked,  or  covered  with  a  thick  black  coating,  is  met  with  oidy  in  se- 
vere cases  in  which  also  the  lips  are  dry  and  fissured,  presenting  bleeding 
rhagades  encrusted  with  a  dark  brown  deposit.  A  foul  odor  issues  from 
the  mouth;  the  bases  of  the  teeth  are  covered  with  a  slimy  }-ellowish 
brown  mass;  and  the  nostrils,  which  the  patients  are  constantly  picking 
as  they  are  their  lips,  appear  ulcerated  anil  incrusted.  On  the  other 
hand,  mycoses,  which  in  the  severe  tj'|)hoid  of  atlults  are  a  frequent  and 
prognostically  bad  symptom,  are  rarely  met  with  in  children. 

Sivelling  of  the  parotid,  according  to  Bicdert,  is  always  indicative  of 
a  severe  mouth  infection  and  a  malignant  course;  it  usually  occurs 
towards  the  end  of  the  second  week,  and  undergoes  suppuration,  pro- 
vided the  patient  lives  long  enough. 


432  THE   DISEASES    OF   CHILDREN 

Pseudomenihrannus  anginas  occur  in  severe  typhoid  fever  develop- 
ing during  tlie  course  of  the  tlisease  anil  rarely  constituting  the  first 
symptoms,  though  I  have  observed  this  in  three  cases  and  it  has  been 
described  by  others.  Of  different  significance  is  a  pharyngeal  affection 
described  by  E.  L.  Wagner  as  "angina  typhosa,"  with  the  development 
of  flat  ulcerations  on  the  palatal  arches  and  likely  to  be  regarded  as  a 
primary  affection.  In  chiklrcn  this  angina  is  met  with  relatively  sel- 
dom, but  instead  of  it  we  frecjuently  notice  a  circumscribed  injection 
affecting  the  palatal  arches  and  the  epiglottis,  with  some  anlema  of  the 
nmcous  membrane.  Mya,  who  studied  more  closely  the  nature  of  an- 
gina mycosa,  was  able  to  cultivate  typhoid  bacilli  from  the  ulcerations 
in  the  pharynx,  which  were  not  present  in  mere  catarrhal  forms. 

Vomiting  is  more  fi-e(juent  than  in  adults,  often  inaugurating  the 
disease  or  accompanying  it.  If  associated  with  constipation, —  an  occur- 
rence by  no  means  rare  in  the  typhoid  fever  of  children, — it  suggests 
meningitis.  Abdominal  pains  are  usually  wanting  or  if  present,  not  vio- 
lent, which  is  in  correspondence  with  the  al)senee  of  intestinal  ulceration. 
Gurgling  in  the  ileocecal  region  is  usually  wanting,  whereas  it  may  be 
found  in  a  large  number  of  diveis  non-typhoidal  intestinal  affections, 
so  that  no  diagnostic  value  can  be  attached  to  it.  Meteorism  is  never 
very  considerable,  sometimes  it  is  absent,  and  at  times  there  may  be 
retraction  of  the  abdomen.  Diarrhma,  as  a  rule,  sets  in  rather  late.  In 
rare  cases,  some  of  which  I  have  observed,  the  disease  begins  with  the 
symptoms  of  a  violent  colitis,  attended  with  tenesmus  and  bloody- 
mucous  stools.  But,  as  already  stated,  in  most  cases  the  thin,  fluid  evac- 
uations in  moderate  number  (3  to  5  in  24  hours)  and  following  a  constipa- 
tion, do  not  appear  until  the  second  week.  Constipation,  however,  may 
continue  throughout  the  course  of  the  disease,  as  I  have  seen  repeatedly. 

The  diarrhwal  discharges  have  the  characteristic,  pea-soup  appear- 
ance, and  if  left  standing  in  a  glass  vessel  present  a  lower  stratum  con- 
sisting of  bright  yellow  and  whitish  flakes.  From  these,  bacilli  may  be 
cultivated  in  doubtful  cases  according  to  the  method  of  Drigalski  and 
Conradi.  An  exceptionally  profuse  diarrha>a  may  cause  the  children  to 
become  very  much  emaciated,  and  it  may  continue  at  most  12  to  14 
days,  though  the  usual  thin  typhoid  stools  are  replaced  much  sooner  by 
solid  evacuations  or  it  may  be  that  constipation  occurs  during  conva- 
lescence. Involuntary  evacuations  are  always  an  unfavorable  symp- 
tom indicating  an  unusually  severe  course  of  the  disease,  especially 
when  succeeded  by  deep  stupor  and  paralysis  of  the  sphincter  so  that 
the  intestinal  contents  steadily  ooze  from  the  gaping  anal  orifice. 

Intestinal  hemorrhages  and  perforations,  with  their  secjuela^  are 
met  with  almost  exclusively  in  older  children  as  in  the  young  there  is 
little  anatomical  alteration,  necrosis  and  ulcerations  being  absent;  but 
when  these  complications  occur  they  have  the  same  dangerous  signifi- 


TYPHOID  FE\ER— ABDOMINAL  TYPHUS  433 

cance  as  in  adult  life.  The  sj-niptoms  of  intestinal  luenionhage  are  sud- 
den collapse,  with  rapid  depression  of  bodily  temperature,  cold  sweat, 
pallor  of  face,  cold  nose  and  extremities,  smallness  of  tlie  pulse,  followed 
in  a  few  hours  or  by  the  next  day  by  the  passage  of  black  or  bright  red 
masses  per  anum.  Perforation  takes  place  only  in  the  later  stages  of 
the  disease  (third  to  fifth  week);  it  begins  with  violent  vomiting  and 
singultus,  to  be  soon  followed  by  collapse  and  a  rapidly  developing, 
painful  peritonitis.  But  peritonitis  may  ensue  without  intestinal  ])er- 
foration  as  a  result  of  an  extension  of  the  process,  by  contiguity,  to  the 
serosa,  and  the  prognosis  in  such  cases  is  less  unfavorable. 

One  of  the  most  important  symptoms  and,  in  doubtful  cases  after 
the  second  year,  of  decisive  aid  in  the  diagnosis  of  typhoid  fever,  is 
swelling  of  the  spleen,  the  frecjuency  of  which  is  about  the  same  as  in 
adults.  The  spleen  enlarges  at  an  early  period  and  grows  steadily  with 
the  development  of  the  fever,  becoming  three  or  four  times  its  original 
size,  particularly  in  its  long  axis.  However,  meteorism  or  overlying  by 
bowel  may  prevent  its  recognition  by  percussion,  while  the  more  relia- 
ble palpation  with  the  hand  and  fingers  flatly  placed  on  the  abdomen 
and  pressing  gently  under  the  costal  arch,  may  be  valueless  either  on 
account  of  the  softness  of  the  spleen  or  the  tension  of  the  abdominal 
muscles.  Certainlv  we  must  never  content  ourselves  with  a  single  exam- 
ination for  the  determination  of  an  enlarged  spleen  is  of  such  decisive 
diagnostic  moment.  During  the  period  of  declining  fever,  the  spleen 
becomes  rapidly  smaller,  usually  attaining  its  normal  dimensions  at  the 
beginning  of  convalescence. 

On  the  part  of  the  respiratory  organs  we  notice  epistaxis  relatively 
seldom  and  only  in  the  early  stages  of  the  process;  except,  however,  in 
those  severe  cases  in  which  it  is  a  phenomenon  of  the  hfemorrhagic  dia- 
thesis. Bronchial  catarrh  is  a  usual  accompaniment  of  typhoid  in 
children,  and  is  localized  mostly  in  the  larger  and  middle  branches  of 
the  bronclii.  Where  expectoration  is  scant  and  cardiac  action  shght,  a 
hypostasis  may  result  in  the  lower  portions  of  the  lung  and,  as  a  result 
of  this,  the  percussion  sound  becomes  duller  and  there  are  moist  rales 
with  soft  bronchial  breatliing  (so-called  sub-broncliial  respiration).  At 
the  same  time  there  occiu-s  a  shght  cyanosis  with  increased  frequency 
of  pulse  and  respiration,  dilatation  of  the  alae  nasi  and  a  call  upon  the 
auxiUary  respiratory  muscles,  symptoms  all  suggestive  of  an  encroach- 
ment on  the  respiratory  area.  A  rise  of  fever  would  occasion  the  suspi- 
cion of  the  development  of  genuine  bronchopneumonic  foci.  Such  an 
occurrence  is  a  serious  comphcation,  at  least  protracting  the  disease, 
and  often  causing  a  fatal  termination  from  asphyxia,  pulmonary  crdema 
or  paralysis  of  the  heart  muscle.  The  development  of  a  croupous  pneu- 
monia may  hkewise  become  a  serious  comphcation  usually  occurring 
at  the  height  of  the  fever  or  at  the  beginning  of  its  dechne.    In  the  latter 

11—28 


434  THE   DISEASES   OF   CHILDREN 

case  the  temperature  \vi\\  again  shoot  upwards,  and  the  pneumonia  may 
become  localized  in  different  portions  of  the  lungs,  especially  in  the  upper 
lobes  and  cause  a  loud  broncliial  breatliing,  typical  crepitant  rales,  se- 
vere dyspnoea,  and  intense  general  phenomena,  or  may  as  a  migratory 
pneumonia  successively  affect  adjoining  portions  of  the  lung.  Pneu- 
monia is  a  serious  compUcation  of  typhoid.  The  form  of  typhoid  fever 
described  by  Gerhal-dt  in  adults  as  "pneumotyphus,"  setting  in  under 
the  aspect  of  a  pneumonia,  followed  by  typhoid  symptoms,  is  very  rare 
in  children.  It  is  onlj'  a  few  weeks  ago  that  I  had  the  opportunity  to 
observe  a  case  of  this  kind  for  the  first  time.  The  patient  was  a  cMld 
twenty-one  months  old  taken  ill  suddenly  with  rapidly  ascending  tem- 
perature, and  a  small  pneumonic  focus  developed  in  the  left  ujjper  lobe, 
which  some  days  later  resolved  without  a  fall  in  the  temperature;  rather 
the  fever  continuing  with  enlarged  spleen,  diarrhoea,  roseola,  in  short 
with  all  the  symptoms  of  a  rather  severe  typhoid  fever  that  terminated 
favorably. 

Gangrene  of  the  lungs  is  a  complication  tliat  rarely  occurs  and  only 
in  intensely  severe  cases.  More  frequently  we  meet  with  pletiritis,  with 
either  serous  or  purulent  exudation,  caused  either  by  the  typhoid  ba- 
cilli or,  more  frequently,  by  a  mixed  infection.  A  latent  tuberculosis, 
localized  in  the  peribronchial  lymph-nodes  may  be  aroused  by  the  fever 
and  especially  by  the  concomitant  bronchial  catarrh,  and  may  mani- 
fest itself  by  a  continuance  of  fever,  which  usually  presents  irregular 
fluctuations  at  times  of  a  hectic  character,  as  well  as  by  increasing  cough, 
dyspnoea,  and  evidence  of  foci  of  infiltration  in  the  lungs. 

Laryngeal  complications,  wliich  in  adults,  especially  in  the  course 
of  certain  epidemics,  are  frequent  and  always  serious  occurrences,  are 
rarely  observed  in  the  tj^phoid  fever  of  children.  They  may  appear  as 
laryngitis,  corresponding  anatomically  to  an  infiltration  of  the  mucous 
membrane;  or  as  ulcerations  analogous  to  those  described  as  occurring 
in  the  pharynx;  or  even  as  a  laryngeal  perichondritis,  ^\^th  cartilaginous 
•necrosis  and  abscess  formation.  Clinically  this  complication  is  mani- 
fest by  aphonia,  rasping  croup-like  cough,  dyspnoea,  fits  of  suffocation, 
and,  if  it  begins  in  the  tliird  or  foiu'th  week,  by  an  increase  of  fever.  A 
form  of  typhoid,  beginning  with  laryngeal  symptoms  and  occurring  in 
children,  has  been  described  by  Schuster  as  "laryngotyphus,"  the 
laryngeal  symptoms  continuing  during  the  whole  course  of  the  disease. 
Fibrinous  inflammation  of  the  larynx  and  paralysis  of  the  laryngeal 
muscles  have  been  reported. 

With  respect  to  cardiac  complications  endocarditis  and  pericarditis 
may  occur,  but  they  are  more  rareh^  encountered  in  typhoid  fever  than 
in  the  com'se  of  other  infectious  diseases. 

Of  the  symptoms  on  the  part  of  the  skin,  the  rose  spots  are  the  most 
conspicuous,  equaling  the  enlarged  spleen  in  diagnostic  value.     They 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  435 

appear  five  to  ten  days  after  the  beginning  of  the  fever,  usually  confined 
to  the  abdomen  and  back,  less  often  except  during  infancy  they  are 
diffused  over  the  entire  body.  The  exanthem  consists  of  pale-red 
slightly  elevated  papules  of  the  size  of  a  pinhead  which  disappear  on 
pressure  \\ith  the  finger.  The  eruption  comes  out  in  .several  .series  and 
vanishes  after  a  short  time.  The  fact  that  the  eruption  cannot  always 
be  easily  demonstrated  is  distinct!}'  indicative  of  typhoid  fever,  the 
often  scanty  efflorescences  must  be  carefully  looked  for  or  they  may 
be  overlooked.  In  a  case  under  my  observation  the  eruption  was  con- 
fined to  a  small  group  of  hchen-hke  nodules  in  the  right  loin.  From 
flea  bites,  which  they' resemble  very  much,  these  efflorescences  are  dis- 
tinguished by  the  absence  of  a  central  point  corresponding  to  the  bite. 
Their  number  is  no  criterion  of  the  com'se  of  typhoid  fever;  but  a  bluish 
color,  instead  of  the  customary  rose,  points  to  a  serious  infection. 
There  are,  however,  cases  in  wliich  the  rose  spots  are  wanting  during 
the  entire  course  of  the  disease. 

Wliile  during  the  first  days  of  the  disease  the  skin  appears  very 
dry,  it  is  possible  that  later,  especially  at  the  initial  remission  of  the 
fever,  a  profuse  perspiration  mth  miliaria  maj'  occur;  this  is  devoid  of 
any  diagnostic  or  prognostic  significance.  Recently  Auche  and  Letrelle 
have  described  cases  of  disseminated  cutaneous  gangrene  which  occa- 
sions bluish  red  plaques  \\ith  ecchymotic  centres  or  abscesses  of  the 
skin.  The  same  author  and  others  have  also  observed  pustules  and 
numerous  cutaneous  and  subcutaneous  abscesses.  I  have  witnessed 
similar  cases,  the  severest  of  which  was  that  of  the  son  of  one  of  my 
colleagues,  whose  whole  body  was  covered  with  numerous  walnut-sized 
pus  foci.  It  is  evident  that  such  compHcations  aggravate  and  protract 
the  disease.  There  occur  also  polymorphous  erythemata,  predomi- 
nantly locahzed  around  the  joints,  and  caused  by  a  micrococcus  closely 
related  to  the  diplococcus  ha?morrhagicus  (Leroux  and  Lorrain). 

Decubitus,  quite  frequent  and  justly  feared  in  adults,  is  rare  in 
children,  and  is  met  with  only  in  the  severe  and  neglected  cases.  The 
decubitus  is  usually  over  the  sacral  bone,  and  never  spreads  extensively 
or  deeply.  Complications  ■nlth  erysipelas  is  an  exceptional  occurrence 
though  it  was  observed  by  Escherich  and  myself,  in  a  case  where  it 
started  from  the  scrotum:  tliis  child  also  presented  evidences  of  idiocy 
and  aphasia.  On  the  other  hand,  herpes  labialis.  which  formerly  was 
regarded  as  negative  of  typhoid  fever,  but  indicative  of  pneumonia,  is 
not  at  all  rare  in  children. 

Paresis  of  the  lower  extremities  is  sometimes  observed.  It  retards 
convalescence,  but  as  a  rule,  it  disappears  ^\ithout  leading  any  perma- 
nent traces.  I  have  already  mentioned  a  case  of  eclampsia  with  sub.se- 
quent  idiocy.  In  such  a  comphcation  we  have  evidently  to  deal  with  a 
localization  of  the  process  in  the  meninges  or  in  the  cerebral  cortex. 


436 


THE   DISEASES   OF   CHILDREN 


Several  authors  have  spoken  of  tlie  demonstration  in  cases  of  menin- 
gitis of  bacilli  in  the  cerebrospinal  fluid  obtained  by  lumbar  puncture. 

Among  the  involvements  of  the  organs  0/  se7ise,  apoplexy  of  the 
retina  described  by  Bouchut  may  be  mentioned,  likewise  otitis  media, 
whose  causation  is  evidently  connected  with  the  mouth  infections  which 
accompany  typhoid  and  are  rather  frequent.  During  the  period  of  con- 
valescence deafness  exists  frequently,  but  is  only  transitory. 

The  blood  exliibits  hypoleucocytosis,  reduction  of  the  amount  of 
haemoglobin  and  of  the  number  of  eosinophilous  elements.  These  find- 
ings may  at  times  be  utilized  for  purposes  of  differential  diagnosis  in 
initial  pneumonia.  I  cannot  substantiate  the  fibrin  reaction  as  stated 
by  Rosenthal  that  no  fibrin  network  forms  in  the  fresh  preparation  of 
typhoid  blood,  whereas  in  pneumonia,  meningitis,  and  other  affections 
with  accompanying  leucocytosis,  a  fibrin  web  may  develop  in  the  course 
of  half  an  hour. 

The  urine  usually  is  scant,  often  contains  albumin,  less  frequently 


Relapses  in  typhoid  fever. 


casts  and  renal  epithelia.  Sometimes  it  exhibits  the  character  of 
nephritic  urine,  and  there  is  a  form  of  the  affection  designated  as 
renal-typhoid  in  which  these  symptoms  manifest  themselves  at  the  very 
beginning  and  dominate  the  disease.  As  already  stated,  typhoid  bacilh 
frequently  occur  in  the  urine.  Ehrhch's  chazo  reaction  usuall}'  proves 
positive  at  the  height  of  the  process.  From  the  time  of  its  appearance, 
its  intensity,  and  duration  we  may,  with  certain  precaution,  draw  prog- 
nostic conclusions.  On  the  part  of  the  sexual  organs  we  observe  rarely 
in  girls  a  pseudomembranous  inflammation  of  the  vagina  and  gangrene 
(noma)  of  the  labia. 

Having  described  the  course  of  hght,  medium  and  mahgnant  cases 
of  typhoid  fever  in  childhood  with  their  comphcations  and  sequels, 
there  remains  to  give  a  short  survey  of  some  particular  and  peculiar 
features  of  the  disease.  Foremost  among  these  are  the  abortive  cases, 
distinguished  from  ordinary  typhoid  by  their  short  duration,  and  accom- 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  437 

panied  either  by  light  or  severe  symptoms.  Next  come  protracted 
cases  in  wliich  without  complications  or  sequelte,  the  fever  may  persist 
for  five  weeks  and  longer.  Finally  we  have  feverless  or  afebrile  cases, 
by  all  means  the  rarest  anomaly  of  the  morbid  process.  These  forms, 
frequently  overlooked  or  falsely  interpreted,  play  a  role  in  spreading  the 
infection  that  must  not  be  underestimated.  Their  recognition  has  been 
materially  facihtated  by  the  modern  methods  of  cultivating  the  bac- 
teria from  the  dejections  and  liy  Widal's  agglutination  reaction. 

Relapses  in  children  are  scarcely  rarer  than  in  adults.  They  may 
occur  during  the  period  of  defervescence,  forcing  the  temperature  again 
upwards;  or  set  in  after  a  brief  afebrile  interval.  The  relapse  may  equal 
in  intensity  and  duration  the  first  onset  or  exceed  it,  or  be  less,  or  repeat 
itself  many  times  (see  temperature  curve  in  Fig.  105)  thus  protracting 
the  duration  of  the  disease  considerably.  In  a  case  reported  by  Comby, 
the  fever  relapsed  six  times  and  lasted  fully  four  months. 

Typhoid  fever  in  infancy  occupies,  in  a  certain  sense,  a  separate 
position.  On  the  strength  of  my  own  experience  I  can  not  confirm  the 
assertion  made  on  various  sides  that  the  affection  is  exceedingly  rare 
during  the  first  half  of  Hfe.  True,  the  symptoms  are  of  a  rather  vague 
nature;  yet  the  course  of  the  temperature  curve,  which  in  point  of  con- 
stancy and  regularity,  is  not  encountered  in  other  febrile  intestinal  affec- 
tions of  this  age,  mil  lead  to  the  right  scent,  and  the  usually  prof  use 
eruption  of  roseola  should  remove  any  doubt.  Marfan,  Gerhardt,  and 
recently  Forget,  consider  the  prognosis  of  the  affection  at  tliis  age  as 
especially  bad,  its  mortaUty,  according  to  the  last-mentioned  author, 
being  50  per  cent.  The  latter  claim,  however,  is  contrary  to  my  obser- 
vation, for  in  a  dozen  cases  of  typhoid  fever  in  infants  there  was  only 
one  with  fatal  termination.  True,  these  infants  were  all  breast-fed, 
which  may  have  a  certain  influence  on  the  prognosis.  Likemse,  the 
extensive  intestinal  alterations  advanced  by  various  -^Titers,  wliich  may 
lead  to  perforation,  I  have  never  been  able  to  observe  in  the  necroscopic 
material  at  the  Prague  Pathological  Institute. 

The  course  and  termination  generally  spealcing  is  hkely  to  be 
shorter  and  more  favorable  than  in  adults,  but  they  exliibit  great  varia- 
tion. The  height  of  the  fever  indicates  the  gra\-ity  of  the  ca.se  to  a  lesser 
extent  than  the  tempestuous  beginning  of  the  phenomena  with  sharp 
ascent  of  the  temperature,  rapidly  developing  disturbance  of  the  sen- 
sorium,  pallor  of  the  face,  dryness  and  fuliginous  coating  of  the  tongue 
and  teeth,  fissured  Ups,  intense  prostration,  feeble  and  frequent  pulse, 
etc.  But  even  in  such  cases  the  conditions  are  not  quite  so  unfavorable 
as  in  later  life  the  two  most  dangerous  contingencies,  intestinal  hemor- 
rhage and  perforation,  being  of  rare  occurrence.  Ambulatory  typhoid 
in  children,  especially  from  the  lower  strata  of  societ}',  is  by  no  means 
rare,  yet  I  have  had  repeatedlj'  patients  from  the  better  classes  brought 


438  THE   DISEASES   OF   CHILDREN 

to  my  office  who  had  been  feverish  for  some  time  and  in  whom  I  was  able 
to  determine  the  fully  developed  disease.  Many  cases  of  this  kind  may 
suddenly  terminate  unfavorably,  as  Biedert  and  others  have  observed. 

The  diagnosis,  on  account  of  its  mild  course  and  the  vague  symp- 
toms during  the  first  week,  is  usually  cjuite  difficult  and  may  be  estab- 
lished only  by  exclusion.  The  course  of  the  temperature,  which  should 
be  taken  every  three  or  four  hours,  the  steadily  increasing  size  of  the 
spleen,  and  the  eruption  of  roseola,  both  of  wliich  symptoms  are  scarcely 
observable  before  the  end  of  the  first  week  of  fever,  finally  clear  up  the 
diagnosis.  However,  very  frequently — and  any  practitioner  of  average 
experience  will  agree  with  me — a  differential  diagnosis  from  other  feb- 
rile conditions  of  cliildhood  may  be  exceedingly  difficult.  Foremost 
among  these  I  mention  mihary  tuberculosis,  which  may  equally  stealth- 
ily set  in,  at  first  presenting  no  local  symptoms  and  with  a  similar 
temperature  curve.  In  such  perplexing  cases  irregular  fluctuation  of 
the  fever  (the  variation  between  morning  and  evening  being  several  de- 
grees), the  absence  of  diarrhoea,  the  presence  of  dyspnoea  with  almost 
negative  pulmonary  findings,  the  relatively  long  duration  of  the  process, 
its  stationary  character,  hereditary  taint,  demonstration  of  tuberculous 
products  in  the  region  of  the  glands  or  in  the  osseous  system,  and  finally 
the  develojjment  of  the  disease  after  measles  or  whooping-cough,  are 
suggestive  of  tuberculosis,  whereas  enlarged  spleen  and  roseola  point 
to  typhoid  fever.  But  even  in  such  cases  mistakes  are  by  no  means  im- 
possible. I,  myself,  for  instance,  observed  a  case  in  which,  immediately 
succeeding  measles,  a  severe  typhoid  fever  developed.  A  positive  diag- 
nosis of  it  was  made  possible  only  after  long  hesitation  and  principally 
on  the  basis  of  its  recovery. 

It  is  under  just  such  conditions  that  the  modern  bacterial  diagnostic 
methods  render  valuable  aid  in  enabling  us  to  differentiate  between 
typhoid  and  tuberculous  meningitis.  Such  differentiation  may  however 
be  attended  with  great  difficulties  at  times,  cases  of  abdominal  fever 
occur  accompanied  by  vomiting,  scaphoid  depression  of  the  abdomen, 
rigidity  of  the  cervical  and  dorsal  muscles,  slow  and  irregular  pulse, 
"cris  cephahques," — in  short,  by  all  symptoms  which  point  to  a  tuber- 
culous meningitis,  and,  on  the  other  hand,  a  tuberculous  meningitis 
especially  in  the  first  years  of  life,  not  infrequently  exhibits  a  course 
like  typhoid  fever. 

In  the  agglutination  test  devised  by  Griinbaum,  elaborated  by 
Gruber  and  Pfeiffer  in  animal  experiments,  adapted  by  Widal  for  chn- 
ical  purposes,  and  subsequently  essentially  improved  by  Fickcr,  we  pos- 
sess a  method  which  in  the  great  majority  of  cases  accomphshes  the 
desired  object  and  into  the  details  of  wliich  I  need  not  enter.  One  of 
the  latest  tests  of  the  procedure  made  by  Hopfengiirtner  in  children 
yielded  a  positive  result  in  all  cases  examined.     The  time  required  for 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  439 

the  observation  has  been  materially  shortened  by  Weil,  assistant  in  the 
Hygiene  Institute  of  the  German  University  in  Prague,  in  the  use  of  a 
test,  heated  to  50°  C.  (122°  F.),  half  an  hour  being  sufficient  to  oiitain 
the  result.  A  small  apparatus  for  the  typhoid  test  is  furnislied  i)y  some 
dealers;  this  enables  the  physician  to  institute  a  diagnosis  conveniently 
at  his  own  residence. 

One  objection  is  the  occasional  late  onset  of  the  reaction,  wliich  is 
rather  frequent  in  children.  Under  such  conditions,  a  diagnostic  exam- 
ination of  the  blood  may  be  required,  making  cultures  either  from 
roseola  or — what  is  best  in  douljtful  cases, — from  the  blood  of  the  bra- 
chial vein  or  the  finger  tip,  according  to  Castellani's  procedure.  Joch- 
mann,  Flamini,  and  Roily  report  unqualified  success  by  this  method  in 
the  great  majority  of  cases  of  typhoid  fever  in  children  examined  by 
them,  during  the  very  first  days  of  the  disease.  Finally  cultivation  of 
bacteria  from  the  stools  may  be  necessary.  According  to  observations 
collected  by  Koch,  the  method  of  Drigalski  and  Conradi  will  quickly 
and  surely  bring  about  a  satisfactory  result  during  childhood. 

The  prognosis  is,  as  a  whole,  favorable  during  childhood,  although 
during  this  period  of  life  grave  cases  and  malignant  epidemics  may  oc- 
cur. Filatow,  a  very  experienced  observer  of  great  clinical  acumen, 
designates  as  unfavorable  prognostic  signs  fuliginous  coating  on  tongue 
and  teeth,  profuse  and  obstinate  diarrha^a,  delirium  in  waking  condi- 
tion (with  eyes  open),  rigidity  of  cervical  and  especially  dorsal  muscles, 
carphology  (picking  the  bed  clothes  with  the  fingers)  thready  pulse  and 
other  phenomena  of  cardiac  weakness,  as  well  as  complete  insensibility. 

Intense  meteorism,  too,  is  a  bad  symptom,  and  constantly  retracted 
abdomen  with  persistently  high  fever  is  yet  worse. 

The  treatment  of  typhoitl  fever,  in  spite  of  accurate  knowdedgc  of 
its  cause  and  of  its  life-peculiarities  has  as  yet  not  reached  any  specific 
method  and  its  principal  task  lies  in  adecjuate  prophylaxis — in  avoid- 
ing the  chief  sources  of  infection  and  as  Koch  suggests  isolating  the  pa- 
tients, and  carefully  disinfecting  their  surroundings,  and  morbid  excre- 
tions. There  can  be  no  doubt  of  the  significance  of  infected  water  and 
soil.  Sanitation  in  large  cities,  consisting  on  one  hand  in  sewerage  and 
(.Irainage  antl  on  the  other  hantl,  in  supplying  wholesome  water  for 
drinking,  bathing  and  culinary  purposes,  has  already  accomplished 
remarkable  results.  Thus,  the  city  of  Munich,  formerly  a  notorious 
haunt  of  typhoid,  has  become  a  salubrious  town,  and,  owing  to  constant 
disregard  for  such  sanitary  arrangements,  Prague  has  for  decades  been 
visited  with  severe  epidemics,  against  which  the  indivi(hial  must  pro- 
tect himself.  Individual  prophylaxis  includes  boiling  and  filtration  of 
water  for  drinking,  cooking,  antl  bathing  purposes;  cleansing  of  vege- 
tables, fruit,  glasses,  etc.,  with  boiled  water;  avoidance  of  bathing  in 
creeks  or  rivers  flowing  through  the  atllicted  locality  whose  waters  niaj' 


440  THE   DISEASES   OF   CHILDREN 

contain  typhoid  bacilli,  careful  cleansing  of  the  hands  of  children  after 
they  have  played  in  dirt — a  series  of  disagreeable  measures  after  all 
and  still  insufficient  for  protection.  Even  a  close  observance  of  these 
precautionary  directions  may  at  times  prove  unsuccessful  in  preventing 
typhoid  fever,  for  either  the  lines  of  defense  were  not  strong  enough  or 
other  sources  of  infection,  unsuspected,  were  left  open. 

In  small  towns,  where  the  conditions  can  be  more  easily  surveyed 
and  the  course  of  the  disease  more  closely  pursued  than  in  the  labyrin- 
thine paths  of  a  metropolis,  strict  isolation  as  recommended  by  Koch 
must  be  insisted  upon,  bacteriological  examination  of  evacuations 
to  be  discontinued  only  when,  after  repeated  observations,  freedom 
from  bacilli  has  been  established,  strict  disinfection  of  dwelling,  etc. 
Brilliant  results  have  already  been  attained  by  following  this  pro- 
phylactic advice. 

In  private  practice,  we  should  isolate  the  patient  and  carefully  dis- 
infect the  stools,  urine,  and  expectorations.  This  is  done  best  and 
cheapest  by  a  copious  addition  of  slaked  lime  to  the  stools  and  urine  and 
of  a  solution  of  sublimate  or  a  concentrated  solution  of  lysol  to  the  sputa; 
by  subjecting  the  underclothing  and  bed  clothes  to  the  action  of  live 
steam;  by  keeping  the  attending  nurses  away  from  other  patients;  by 
scrupulous  cleansing  of  the  hands,  etc. 

Before  dealing  with  the  still  necessary  symptomatic  treatment,  I 
shall  briefly  review  the  results  of  specific  therapy.  Pfeiffer  and  Kolle 
availed  themselves  of  an  active  immunization  method,  injecting  agar 
cultures  of  the  typhoid  bacillus  which  hail  been  floated  by  a  solution  of 
common  salt  and  killeil  by  heating.  A  similar  procedure  employed  by 
Wright  with  British  soldiers  in  India  is  said  to  have  been  attended  with 
success.  The  general  harmlessncss  of  this  immunizing  method,  as  con- 
firmed on  various  sides,  justifies  its  trial  in  severe  and  widespread 
typhoid  epidemics. 

Chantemesse  proposed  a  serum  treatment.  For  this  purpose  he 
uses  serum  from  horses  immunized  by  gradually  increased  injections  of 
a  typhoid  toxin  that  he  prepared.  In  patients  thus  treated  he  had  a 
mortality  of  6  per  cent,  and  it  seemed  that  the  process  of  the  disease  was 
milder  and  shorter.  Josias,  among  50  cases  treated  with  this  serum, 
recorded  only  two  deaths,  and  with  early  injections  he  produced  an  abor- 
tive course  and  never  experienced  any  unpleasant  after  effects.  For  my 
own  jmrt,  I  have  not  yet  tested  this  treatment. 

Jez  prepares  a  sort  of  pulp  from  the  bone  marrow,  spleen,  thynuis, 
brain,  and  spinal  marrow  of  rabbits  highly  immunized  against  typhoid; 
crushing  the  pulp  in  a  mortar,  and  adding  a  mixture  of  alcohol,  common 
salt,  and  water,  stirring  up  the  mass,  placing  it  into  an  ice  chest  for  24 
hours,  and  finally  filtering.  The  rather  clear,  reddish  yellow  filtrate  is 
administered  by  mouth.     In  its  use,  Jez  noticed  a  rapid  fall  of  the  tern- 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  441 

perature  and  speedy  improvement  of  the  symptoms.  Results  communi- 
cated from  other  sources,  however,  are  contradictory.  I  used  it  only 
once,  as  prepared  in  Tavel's  laboratory  in  Berne  (Switzerland).  The 
case  was  a  girl  eight  years  old  suffering  from  severe  typhoid  fever.  Two 
of  her  younger  sisters  had  the  disease  but  with  symptoms  less  intense. 
The  treatment  did  not  shorten  the  morbid  process,  nor  influence  the 
temperature  curve  nor  even  prevent  a  relapse.  Still,  I  am  not  inclined 
to  pronounce  judgment  on  the  strength  of  a  single  case. 

As  to  symptomatic  treatment,  not  much  must  be  expected.  I  am 
sure  from  a  rather  wide  experience  in  Prague  in  the  treatment  of  typhoid 
cases  that  none  of  the  many  antipyretic  nor  antiseptic  methods  either 
in  my  own  practice  or  in  that  of  others  presented  anything  to  convince 
me  of  its  efficiency.  Some  of  these  modes  of  treatment  are  disagreeable 
to  the  patient,  and  some  distinctly  dangerous  and  for  such  reasons  a 
wise  restriction  of  their  use  cannot  be  too  strongly  recommended. 

The  hope  of  sharply  checking  the  process  by  energetic  primary 
intestinal  disinfection  gave  rise  to  the  calomel  treatment.  Apart  from 
the  fact  that  the  object  in  view  cannot  be  attained  by  any  remedy  and 
that  for  the  most  part  we  see  the  cases  at  a  stage  when  the  blood  circu- 
lation has  already  been  colonized  by  the  bacilli,  the  above  method  is  not 
without  certain  dangers,  as  it  irritates  the  bowel  and  is  apt  to  provoke 
stomatitis  and  ulceration  of  the  gums.  For  the  benefit  of  the  patients, 
it  is  better  not  to  use  it.  Whoever  wants  to  use  any  of  the  "intestinal 
antiseptics,"  as  salol,  benzonaphthol,  etc.,  will  at  least  not  cause  any 
harm.  They  are  administered  in  the  form  of  a  powder  or  emulsion  in 
daily  doses  of  0.5-2  Gm.  (7^-30  gr.),  according  to  age. 

In  profuse  diarrhoeas  astringents  are  indicated.  Among  these  are 
subnitrate  of  bismuth,  tannalbin,  tannigen,  fortoin,  enterorose,  bismu- 
tose,  ichthalbin,  in  doses  of  0.1-0.25  Gm.  (U-4  gr.),  with  or  without 
addition  of  opium,  a  knife-pointful  of  these  powders  three  to  four 
times  daily.  The  ordinary  typhoid  diarrhoea  with  2  to  4  evacuations 
a  day  is  best  when  unchecked.  The  strenuous  may  try  to  remove 
a  part  of  the  infectious  material  by  the  high  injections  as  recommended 
by  Marfan. 

Against  the  fever  the  whole  arsenal  of  the  antipyretic  method  used 
to  be,  and  is  by  many  still  called  into  requisition,  not  for  the  benefit  of 
the  children,  but  as  statistics  show  at  times  to  their  harm,  as  in  collapse 
from  cold  baths,  or  after  large  doses  of  antipj'retics,  and  rarely  to  their 
joy,  as  can  be  inferred  from  the  excitement  caused  by  anj'  of  the  hydro- 
pathic measures  in  these  poor  little  sufferers.  It  is  my  firm  conviction 
gained  in  the  course  of  many  years  from  the  unprejudiced  observation 
of  numerous  cases,  that  the  progress  of  abdominal  typhoid  in  cliildhood 
is  neither  shorter,  nor  milder,  nor  more  pleasant  for  the  cliildren,  if  tlie 
temperature,  according  to  one  or  another  method,  is  artificially  reduced. 


442  THE    DISEASES   OF   CHILDREN 

The  appetite  of  the  httle  ones  is  not  increased  either  l)y  the  baths  or  by 
antipyretics  administered  internally,  but  it  returns  when  the  fever 
naturally  declines,  because  the  infection  has  exhausted  itself,  the  body 
has  asserted  its  mastery,  and  the  toxin  which  paralysed  the  digestive 
functions  is  formed  and  absorbed  no  more. 

Above  all  and  most  em]ihatically,  I  would  caution  against  the 
strict  observance  of  such  antipyretic  measures  as  Brandt's  or  Vogl's, 
who  have  gained  for  themselves  as  unenviable  remembrance.  Such 
coarse  methods  (I  cannot  find  a  milder  designation  for  them)  are  apt  to 
produce  such  disagreeable  sensations  that  the  patients  do  not  crave  for 
their  repetition. 

In  case  of  severe  disturbance  of  the  nervous  system,  especially 
insomnia,  a  warm  bath — about  35  to  36°  C.  (95°-97°  F.)  and  given  in 
the  presence  of  the  physician — may  be  serviceable,  and  if  there  be  any 
stupor,  water  of  the  room  temperature  may  be  poured  on  the  head. 
During  the  last  few  years  I  have,  under  such  contlitions  when  the  heart 
action  was  good,  prescribed  small  doses  of  pyramidon,  0.1  to  at  most 
0.15  Gm.  (H  to  2  gr.),  administered  once  in  the  evening.  The  reduction 
of  the  temperature  effected  thereby  is  gradual,  but  lasting  for  a  long 
time,  and  the  sootliing  effect  is  undeniable. 

Other  than  tliis  I  use  only  hydropathic  comjjresses  with  slightly 
heated  water,  changing  them  every  three  hours  and  covering  them  with 
a  dry  cloth.  In  case  they  should  give  rise  to  any  unpleasant  sensations 
or  excite  the  cliild  I  simply  dispense  with  them.  Apart  from  the  fact 
that  the  little  patients  generally  stand  fever  very  well  and  often  wth  a 
temperature  of  39°  C.  (102°  F.)  and  above,  will  .sit  upright  in  their  beds 
and  play,  they  do  not  feel  any  better  when  their  bodily  temperature  has 
been  artificially  reduced. 

Nutrition  is  of  importance  and  of  course,  during  the  whole  period 
of  fever  the  diet  must  be  Uquid  and  such  as  milk  or  in  case  of  dishke  of 
milk,  coffee,  tea,  cocoa,  soups,  eggs,  egg  punch  (on  account  of  its  alco- 
hohc  contents  indicated  when  the  pulse  is  small).  To  increase  their 
nutritive  value,  somatose,  plasmon,  tropon,  Leube's  meat  solution,  puro, 
and  the  hke  may  be  added.  They  should  be  given  in  small  quantities 
and  at  frequent  intervals  as  the  patient  will  not  take  much — every  one 
and  a  half,  two  to  three  hours — and  also  abundant  drink  such  as  boiled 
sterilized  water,  lemonade,  light  natural  acidulated  waters,  etc. 

When  there  is  vomiting  or  deep  stupor  makes  the  taking  of  food  by_ 
mouth  impossible,  enemata  may  be  tried,  made  of  eggs,  flour,  milk,  and 
salt,  or  in  the  form  as  recommended  by  A.  Schmidt  ready  for  use  and 
steriHzed  (made  by  Heyden  of  Radebeul  near  Dresden). 

Great  loss  of  water  as  a  result  of  profuse  tharrhoca  should  be  equal- 
ized by  subcutaneous  infusions  of  common  salt,  and  waning  heart  power 
strengthened  by  bold  alcoholic  administration  in  the  form  of  mild  dessert 


TYPHOID  FEVER— ABDOMINAL  TYPHUS  443 

wines  or  champagne,  injections  of  ether,  camphor,  and  the  hke.  Com- 
phcations  involving  the  lungs  require  expectorants  or  inhalations  of 
oxygen  and  the  attempt  may  be  made  to  check  intestinal  hjEmorrhages 
by  injections  of  gelatin — two  to  five  per  cent.,  sterilized,  in  doses  of  40 
to  80  c.c.  (li  to  3  oz.)  according  to  age.  Intestinal  perforation  has 
recently  been  successfully  operated  upon  by  Stewart. 

Frequent  cleansing  of  the  mouth  to  prevent  or  restrict  secondary 
infections  is  very  much  to  be  recommended.  This  may  be  done  either 
by  wasliing  out  the  mouth  with  a  piece  of  gauze  dipped  in  boric  acid 
solution  or  by  repeated  rinsing  and  gargling.  Good  service  is  rendered 
also  by  menthol  vaseline  (0.5  to  1  per  cent,  with  vaseUne  oil)  instilled 
into  the  nose  twice  a  day. 

Scrupvlous  cleanliness,  especially  after  each  evacuation,  is  the  best 
means  for  preventing  decubitus;  also  a  smooth  firm  matress  and  fre- 
quent change  of  position.  There  must  be  an  ample  supply  of  fresh  air 
wliich  can  best  be  procured  when  conditions  permit  bj^  ha^^ng  two 
rooms  at  the  patient's  disposal  which  can  be  alternately  ventilated  and 
occupied.  If  a  mixed  infection  is  present  it  must  be  treated  locally;  in 
case  of  pus  foci,  they  should  be  opened  and  protected  by  bandages. 
The  appetite  may,  vnth  the  defervescence  of  the  fever,  recur  with  vigor. 
but  its  premature  gratification  by  solid  food  should  be  sternly  refused; 
the  return  of  the  appetite  while  undeniably  welcome  is  vmder  such  con- 
ditions rather  perplexing.  We  must  make  a  firm  stand  against  soft- 
hearted attendants  and  absolutel)^  forbid  all  solid  food  such  as  soft 
rolls,  meat  hash,  etc.  Such  food  should  be  \Aithheld  until  about  a  week 
after  complete  disappearance  of  the  fever,  or  even  somewhat  longer  in 
case  the  disease  has  been  a  severe  one.  After  another  week  the  cliildren, 
who,  in  the  meanwliile  have  been  out  of  bed  for  three  to  four  days,  may 
be  allowed  out  to  drive  when  the  weather  permits.  ^Miere  conditions 
are  favorable,  a  stay  in  the  country  during  convalescence  is  to  be 
recommended.  Return  to  school  must  not  be  permitted  until  after 
complete  physical  and  mental  recuperation. 


DYSENTERY 

BY 

Dr.  J.  LANGER,  of  Prague 

TRANSLATED    BY 

Dn.  FRANK  X.  WALLS,  Chicago,  III. 


Dysentery  is  one  of  the  diseases  longest  known,  and  may  be 
defined  as  an  infectious  disease  localized  especially  in  the  colon  and 
appearing  either  endemically  or  epidemically,  the  principal  clinical 
symptoms  of  which  are  tenesmus,  bloody  mucus  stools,  abdominal  pain, 
and  early  prostration. 

Although  the  clinical  picture  of  the  disease  has  been  enlarged  by 
abundant  casuistic  material,  and  histological  examinations  have  cleared 
up  the  details  of  the  pathologico-anatomical  processes  in  the  bowel,  yet 
the  etiology  was  shrouded  in  obscurity  until  the  last  few  years.  How- 
ever, recent  investigations  have  resulted  in  commendable  success.  Thus 
it  has  been  established  by  KartuHs,  Lutz,  Councilman,  and  others,  that 
tropical  dysentery  is  caused  by  a  parasitic  protozoa,  the  amoeba,  and 
in  our  latitudes  these  parasites  seem  to  have  an  occasional  etiological 
significance,  according  to  Losch,  Alva,  Kovacs,  Quincke,  and  others. 
More  frequently,  however,  the  infection  is  caused  by  the  bacillus  of  dys- 
entery which  has  been  cultivated  from  the  evacuations  by  Sliiga,  Kruse, 
and  Flexner  in  epidemics  of  dysentery  in  various  localities.  According 
to  the  investigations  made  by  these  authors,  and  from  a  considerable 
number  of  later  investigations — (the  literature  bearing  on  this  subject 
has  been  carefully  reviewed  by  0.  Lentz  and  Leiner) — there  exist  sev- 
eral varieties  of  dysentery  bacilli,  which  may  be  distinguished  from 
each  other  not  only  culturally  but  particularly  by  serum  diagnosis. 
Further  studies  must  be  undertaken  to  clear  up  the  question  touched 
by  many  authors  as  to  the  relation  of  follicular  enteritis  in  childhood  to 
infectious  dysentery.  Children  were  the  material  used  as  a  basis  for  the 
observations  of  Leiner  and  Jehle. 

Pathology. — The  pathologico-anatomical  findings  depend  upon 
the  intensity  of  the  local  process  as  well  as  the  duration  of  the  disease. 

In  the  mild  cases  wliich  recover  within  a  few  days  there  are  likely 
to  be  circumscribed  areas  of  redness  and  oedema  of  the  mucous  mem- 
brane of  the  colon,  accompanied  by  epithelial  necrosis,  sometimes  by 
shallow  ulcerations,  while  in  cases  characterized  by  greater  intensity  we 
find  flocculent  deposits  or  firmly  adherent  grayish    white  or  greenish 

444 


PLATE  23. 


Sigmoid  flexure  in  dysentery  (lUyear-oid  child). 

Ascending  colon  in  dysentery  (same  child\ 

Bloody  and  slimy  stool  in  follicular  enteritis  (dysentery-like  case)Cphotograplied  from  nature). 


DYSENTERY  445 

yellow  membranes  on  the  mucosa,  which  is  strongly  injected  and  ocdoma- 
tous,  with  here  and  there  haemorrhagic  infiltration.  When  these  deposits 
and  exudates  have  desquamated,  there  results  an  ulceration  which  va- 
ries in  size,  sometimes  isolated,  sometimes  confluent,  or  even  areas  of 
ulcerations  that  extend  more  or  less  deeply  into  the  intestinal  wall  and 
may  corrode  even  the  larger  blood  vessels.  The  intestinal  wall  through- 
out is  tliickened,  edematous,  infiltrated,  and  the  solitary  foUicles  are 
more  or  less  swollen  and  their  surfaces  at  times  ulcerated  (see  Plate  23). 
The  serosa  over  the  affected  intestine  appears  dull  and  lustreless,  and 
the  regional  mesenteric  lymph-glands  are  swollen  and  frequently  infil- 
trated with  blood.  Besides  the  colon,  the  cecum  and  even  the  lower 
part  of  the  ileum  may  become  the  seat  of  these  pathological  alterations. 
The  spleen  is  usually  greatly  swollen,  while  the  Hver  and  kidneys  are 
acutely  degenerated. 

Symptomatology. — The  chsease,  as  a  rule,  begins  like  an  intestinal 
catarrh,  witli  profuse,  diarrhoeal  evacuations.  In  one  or  two  days  later 
tenesmus  occurs  during  and  after  the  evacuation.  Children  affected 
with  the  chsease,  moaning,  bearing  down  and  with  a  painful  expression 
on  their  faces,  usually  tarry  a  long  time  on  the  commode  and  are  loath 
to  leave  it.  The  quantity  of  a  single  evacuation  often  amounts  only  to 
one  or  two  spoonfuls  of  at  first  a  glassy  mucus,  but  later  on  consists  of 
a  mucopurulent  mass  containing  small  streaks  or  even  small  clots  of 
blood,  and  occasionally  dense  flocculi  or  even  membranes.  The  pecu- 
liar odor  characterizing  the  early  mucus  stools  is  soon  displaced  by  a 
carrion-hke  fetor.  The  latter  is  due  to  the  putrefaction  of  extravasated 
blood,  or  it  may  indicate  a  severe,  even  gangrenous  inflammation  of  the 
bowel.  The  number  of  evacuations  during  twenty-four  hours  fluctuates 
between  10,  20  or  maybe  50  and  even  more. 

The  abdomen,  for  the  most  part,  is  depressed,  so  that  on  palpation 
the  contracted  colon  may  be  frequently  felt.  In  such  a  case  the  bowel, 
either  along  the  whole  tract  or  in  circumscribed  localities,  manifests 
more  or  less  acute  sensitiveness  to  pressure.  The  tissue  about  the  anus 
is  usually  very  much  reddened,  often  excoriated,  or  even  ulcerated, 
while  in  the  gaping  anus  may  be  seen  the  tenseh'  filled  veins  and  a  chap- 
let-hke  pad  of  livid,  discolored  mucous  membrane.  The  constitutional 
symptoms  soon  become  manifest.  The  coUcky  pains  that  precede  and 
accompany  the  evacuations  with  the  consequent  tenesmus  torment  the 
patient  not  less  than  the  intense  thirst.  The  sufferer  is  deprived  of 
sleep,  or  sleeps  only  Ughtly.  Even  a  few  days  after  the  disease  has  set 
in  the  patient's  face  exliibits  a  painful  expression,  the  eyes  are  circled 
with  blue,  the  Ups  are  usually  dry  and  fissured,  the  tongue  dry  and 
thickly  coated,  the  appetite  is  gone,  and  often  there  exist  nausea  and 
vomiting. 

It  is  distinctly  characteristic  of  dysentery  that  witliin  a  few  days 


446  THE    DISEASES   OF   CHILDREN 

the  skin  becomes  very  pale  and  there  is  a  rapid  loss  of  strength  and  great 
emaciation.  The  urine  is  usually  lessened  in  amount  and  may  contain 
albumin  and  casts.  The  temperature  presents  notliing  characteristic. 
It  may  be  normal  or  subnormal,  but  in  the  majority  of  cases  it  exhibits 
an  irregular  remittent  type. 

In  a  microscopical  examination  of  the  stools  we  find,  in  and  around 
the  structureless  mass  of  mucus,  intestinal  epitheUa,  single  and  grouped 
leucocytes,  which  are  usually  polynuclear;  erythrocytes  normally  colored 
or  shadowed,  often  agglutinated,  the  occasional  remnants  of  vegetable 
or  animal  food  and  remarkably  few  bacteria.  Concerning  the  bacteria 
it  may  be  stated  that  in  a  cover-glass  preparation  the  presence  of  a  few 
short,  plump,  free  or  endocellular  baciUi,  negative  to  Gram's  stain  with 
many  pus  corpuscles  may  strengthen  our  suspicion  as  to  an  infection  by 
dysentery  bacilh.  But  a  further  identification  of  the  latter  is  possible 
only  by  means  of  cultures  or  finally  by  serum  diagnosis. 

The  progress  and  termination  of  dysentery  vary,  a  complete  return 
to  health  in  a  majority  of  the  cases  ensuing  in  a  more  or  less  short  (1  to 
2  weeks)  or  long  time  (3  to  4  weeks).  But  the  convalescence  of  the 
patients  may,  without  any  manifest  cause  be  interrupted  by  one  or  more 
relapses.  Cases  which  are  grave  or  very  severe  from  the  outset  may 
terminate  fatally  within  a  few  days,  owing  to  a  collap.se  or  various  com-- 
plications.  Sighs  of  favorable  trend  are  remission  of  tenesmus,  the 
occurrence  of  stools  of  a  feculent  odor  and  of  flatus,  decrease  of  tliirst, 
refreshing  sleep,  and  return  of  the  appetite. 

Cases  continuing  for  several  weeks  or  several  months,  in  which 
periods  of  improvement  and  apparent  cure  alternate  ^^^th  relapses,  are 
usually  designated  as  chronic  dysentery.  Not  infrequently  such  cases 
occasion  a  severe  marasmus  or  certain  sequelae  or  comphcations  may 
lead  to  death. 

The  following  complications  of  dysentery  have  been  observed:  Se- 
vere thrush,  stomatitis  either  aphthous  or  ulcerative,  noma,  suppura- 
tive parotitis,  icterus,  hver  abscesses,  peritonitis,  fissures  of  the  anus, 
prolapse  of  the  anus  and  rectum,  gangrene  of  the  prolapsed  anus,  bron- 
chitis, bronchopneumonia,  pneumonia,  atelectasis,  pleuritis,  pya-mia, 
obstinate  tendinous  and  articular  inflammations. 

As  sequelae  there  have  been  recorded:  chronic  cohtis,  membranous 
enteritis,  stricture  of  the  anus,  of  the  rectum  and  of  the  colon,  distur- 
bance of  the  nerves  of  the  lower  extremities,  auEemia  and  marasmus. 

The  diagnosis  in  a  majority  of  cases  of  infectious  dysentery  is  easy; 
the  intestinal  symptoms  and  the  examination  of  the  stools,  especially 
in  an  endemic  or  an  epidemic  of  the  disease,  being  sufficient.  More 
difficult,  however,  is  the  etiological  diagnosis  of  a  sporacUc  case,  as  well 
as  the  differential  diagnosis  of  severe  cases  of  folUcular  enteritis  wliich 
may  be  caused  by  infection  with  highly  virulent  colon  bacteria  (Rossi- 


DYSENTERY  447 

Doria,  1892,  Escherich,  1895,  Finkelstein,  1896).  In  such  contingencies 
an  exact  etiological  diagnosis  is  possible  only  by  means  of  culture  and 
serum  reaction. 

The  prognosis  depends  on  the  intensity  and  extent  of  the  local 
process,  the  conipUcations,  and  the  constitution  of  the  patient.  The 
mortahty  in  several  epidemics  has  fluctuated  between  five  and  thirty 
per  cent. 

With  regard  to  the  prophylaxis,  the  cases  of  dy.?entery  must  be  iso- 
lated both  in  private  practice  and  in  the-hospitals,  the  evacuations  must 
be  disinfected,  and  the  attendants,  both  for  their  own  interest  and  that 
of  those  around  them,  must  be  scrupulously  clean. 

Treatment. — As  to  the  treatment,  the  dysentery  patient  should  be 
confined  to  bed,  even  if  the  disease  be  only  hght.  Warm  compresses,  in 
moist  or  dry  form,  apphed  to  the  abdomen,  are  appreciated  by  most  suf- 
ferers. The  diet  should  consist  of  mucilaginous  soups  made  of  oatmeal  or 
flour,  and  later  on  may  be  given  gradually,  milk,  gruel  soups,  eggs, 
purees,  and  minced  meat.  To  reUeve  thirst,  tepid  tea,  coffee,  pure  water, 
or  sugared  water  to  which  some  brandy  or  a  few  spoonfuls  of  red  wine 
have  been  added,  are  ad^-isable.  In  weakness  or  collapse  cognac  or 
medicinal  wines  (Mavrodaphne,  St.,  Maura,  Sherry,  etc.)  in  large  doses, 
should  be  administered,  and  injections  of  camphor  in  oil  (camphor  1 
part  in  9  parts  of  oHve  oil)  may  be  given  several  times  a  day.  i-1  c.c. 
(m,  7J-15)  with  a  Pravaz  syringe.  For  the  same  purpose  a  subcutaneous 
injection  of  150  to  250  c.c.  (5  to  8  oz.)  of  0.8  per  cent,  solution  of 
chloride  of  sodium  can  be  recommended. 

After  each  bowel  movement,  the  anus  and  the  adjoining  parts 
should  be  cleansed  with  w'ater,  and  then  powdered  or  coated  with  vase- 
line. Medicinal  treatment  should,  wiienever  possible,  begin  with  an 
evacuation  of  the  bowels.  For  tliis  purpose  the  salines  or  castor  oil  is 
given;  of  the  latter,  according  to  the  cliild's  age,  a  teaspoonful  or  table- 
spoonful  is  given  every  half  hour  or  hour,  until  a  stool  follows  and  the 
oil  appears  in  the  excreta.  As  castor  oil  is  thick  and  viscous,  the  spoon 
should  be  heated  over  a  candle.     The  following  emulsion  is  a  favorite: 

a   Olei  ricini 10-15-25 .oi'-S^"' 

Ad  emiilsionem   spl 90 ,5  iii 

Glycerini 5 3  i 

Sig. — -A.  tea.spoonful  to  tablespoonful  every  half  hour  or  hour  until  desired  result 
is  obtained. 

Calomel  is  apt  to  provoke  tenesnuis  or  increase  enormously  that 
already  existing  and  cannot  be  recomnieniled  for  dj'sentery. 

Tenesmus  may  frequentlj*  be  alle\iated  by  warm  compresses  ap- 
plied to  the  perineum,  or  by  an  enema  of  20  to  50  c.c.  of  water  of  the 
same  temperature  as  the  body  2  to  3  times  a  day,  or  an  amylaceous 
decoction  (1  teaspoonful  of  starch  to  1  htre  of  water),  with  later  addi- 


448  THE   DISEASES   OF   CHILDREN 

tion  of  tincture  of  opium — one  drop  for  a  two-year-old  child;  two  drops 
for  a  three-year-old;  three  drops  for  a  four-year-old,  etc.,  in  the  24 
hours. 

Often  suppositories  have  to  be  resorted  to  and  may  be  introduced 
twice  a  day.  As  ana?sthetics  may  be  recommended  opium  or  ana»sthesin, 
the  latter  in  doses  twice  as  large  as  the  former. 

R     Extr.  opii  aquosi  0.005 t2  gr f'"'  two-year-old  child. 

0.01 5  gr for  tliree-year-old  child. 

0.02 j  gr for  five  to  six-year-old  child. 

Butyri  cacao  q.  s.  ut.  fiat  supp.  minus. 
Dentur  talia  suppos.  No.  IV. 
Sig. — Introduce  one  or  two  daily. 

Local  cold  applications  are  also  serviceable.  One  may  introduce 
into  the  anus  a  piece  of  pure  ice  of  the  size  of  a  cherry  every  5  to  10 
minutes  for  half  an  hour  to  one  hour. 

After  the  cleaning  of  the  bowel  one  may  give  internally  subnitrate 
of  bismuth  several  times  a  day.     For  instance,  every  three  hours: 

0.1 gr.  iss for  a  child  half  a  year  old. 

0.2 gr.  iii for  a  child  one  year  old. 

0.3 gr.  ivss for  a  child  two  years  old. 

0.4 gr.  vi for  a  child  tliree  years  old,  etc. 

The  bismuth  may  be  given  also  in  emulsion,  to  be  well  shaken  before 
taking. 

R      Bismuthi  subnitr.  ...  0..5 gr.  viiss for  child  one-half  year  old. 

Aqua;  destillatce  ...  .70 f  §ii  giiss. 

Mucil.  gum.  arab.  . .  .20 3  v. 

Syr.  spl 10 3iiss. 

Sig. — A  teaspoonful  every  three  hours.    Shake  well. 

Instead  of  the  bismuth  salt,  the  bismuth  albumin  combination, 
bismutose,  may  be  given  in  doses  about  three  times  as  large. 

As  internal  astringents,  tannalbin  or  tannigen  may  be  used  three 
to  four  times  daily,  in  doses  of  0.3  to  0.5  Gm.  (4J-7i  gr.).  Their  combi- 
nation with  doses  of  bismuth  corresponding  to  the  age  of  the  child  is 
quite  beneficial.  In  persistent  fever  tannate  of  quinine  renders  good 
service.     Give  three  to  four  times  a  day: 

0.03  to    0.05 gr.  ss-i to  a  child  one-half  year  old. 

0.1 gr.  iss   to  a  child  one  year  old. 

0. 15 gr.  ii to  a  child  two  years  old. 

0.2 gr.  iii    to  a  child  three  years  old. 

0 .  25 gr.  iv    to  a  child  four  to  five  years  old. 

Local  treatment  of  the  intestinal  process  with  irrigations,  although 
appearing  theoretically  advantageous,  proves  usually  too  irritating  at 
the  initial  stage  of  the  disease  and  must  therefore  be  reserved  for  a  later 
period.     One  may  use  enemata  containing  \  to  1  per  cent,  of  table  salt 


DYSENTERY  449 

and  the  cleansing  injection  may  be  followed  by  the  introduction  of  an 
astringent,  the  quantity  of  wliich  must  never  exceed  150  to  200  c.c. 
The  astringents  used  are: 

0.5  to  1  per  cent,  solution  of  tannic  acid. 
1  to  2  per  cent,  solution  of  liquor  aluminum  acetate. 
•  0,1  per  cent,  solution  of  nitrate  of  silver. 

The  last  mentioned  should  always  be  followed  by  a  second  irriga- 
tion with  a  weak  solution  of  common  salt.  They  should  be  made  daily 
or  every  other  day.  In  protracted  cases  I  have  found  efficacious  the 
injection  daily  or  every  other  day  of  an  emulsion  of  bismuth  in  a  muci- 
laginous veliicle. 

1  to  2 gr.  xv-xxx subnitrate  of  bismuth  for  children  two  to  three  years. 

3  to  4 gr.  .xlv-5i subnitrate  of  bismuth  for  children  four  to  five  years. 

5  to  8 gr.  3i-ii subnitrate  of  bismuth  for  children  over  five  years. 

Given  in  100  Gm.  (3  oz.)  mucilage,  gum  arable,  etc.  But  such  an 
enema  should  always  be  preceded  by  a  cleansing  injection.  Complica- 
tions and  sequelae  that  were  previously  mentioned  require  an  individual 
symptomatic  treatment. 

Further  observations  are  needed  before  the  specific  serum  therapy 
of  dysentery  as  estabhshed  through  animal  experimentation  by  Kruse, 
Shiga,  Rosenthal  and  Kanel  and  already  chnically  tested,  can  be  intro- 
duced into  general  practice.  It  is  only  quite  recently  that,  Liidke  has 
affirmed  the  favorable  effect  produced  by  Kruse's  dysentery  serum. 


11—29 


INFLUENZA 

BY 

Dr.  J.  H.  SPEIGELBERG,  of  Munich 

TRANSLATED     BY 

Dr.  henry  L.  K.  SHAW,  Albany,  N.  Y. 


Influenza  has  the  same  importance  in  childhood  as  in  adult  hfe 
and  it  is  worthy  of  special  study  because  it  exhibits  a  number  of  char- 
acteristic and  important  modifications. 

Influenza  is  known  to  have  been  prevalent  in  the  fourteenth  century, 
but  a  cHnical  and  scientific  knowledge  of  the  cUsease  was  not  obtained 
until  the  epidemics  of  1S30  and  1S40.  A  great  advance  in  our  knowledge 
of  the  disease  was  made  during  the  great  pandemic  of  1889  to  1891. 

Influenza  (epidemic  grippe)  is  an  acute  contagious  and  infectious 
disease  occurring  mostly  in  epidemics  and  its  cause  is  a  specific  organ- 
ism, the  bacillus  of  influenza. 

R.  Pfeiff'er  discovered  this  bacillus  in  1889  after  a  number  of  false 
observations  had  been  pubhshed.  His  work  has  been  verified  by  re- 
peated investigations  so  that  to-day  the  importance  of  Pfeiffer's  bacillus 
of  influenza  is  no  longer  in  question.  It  is  a  small,  rod-shaped  bacillus, 
strongly  anaerobic,  with  very  shght  resistive  power  and  short-hved  out- 
side the  body.  Its  vitaUty  is  soon  destroyed  in  water  and  in  earth,  and 
for  cultivation  it  must  be  grown  on  blood  haemoglobin  at  a  temperature 
between  26°-43°  C.  (76°-106°  F.).  The  contagion  is  spread  from  the 
secretions  of  the  mucous  membranes.  The  bacillus  is  found  in  vast 
numbers  in  the  nasal  secretions,  while  in  an  ordinary  coryza  very  few 
bacilli  are  present.  At  first  the  bacilli  are  found  free  in  the  secretion, 
but  later  inside  the  pus  cells.  For  a  rapid  bacteriological  diagnosis  it  is 
best  to  make  smears  from  the  nasal  secretion  and  stain  with  a  weak 
carbol-fuchsin  solution.  In  cliildren  the  bacillus  has  been  found  in  the 
blood,  various  secretions,  cerebrospinal  fluid,  in  pus  from  the  ears,  etc. 

The  incubation  period  differs  according  to  different  authorities  from 
one  to  eight  days. 

The  many  variations  in  the  chnical  picture  of  tlris  disease  are  due  to 
the  fact  that  there  is  an  infectious  inflammation  of  chssimilar  organs 
and  tissues. 

The  parts  most  affected  are  the  respiratory  mucous  membranes, 
through  wliich  the  bacillus  gains  entrance  into  the  system,  the  ahmen- 
tary  tract,  and  the  nervous  system  as  a  result  of  toxic  irritation. 

450 


INFLUENZA 


451 


The  predominance  of  various  symptoms  depends  on  the  part  spe- 
cially affected.  The  symptoms  may  be  loosely  grouped  into  those  of 
catarrhal,  gastric  and  nervous  (rheumatoid)  influenza  and  at  times  into 
a  combination  of  all  three. 

In  a  widespread  epidemic  no  age  escapes,  although  it  is  compara- 
tively rare  in  early  infancy.  It  occurs  most  frequently  from  fifteen  to 
forty  years  of  age.  The  ages  of  47,000  cases  treated  by  physicians  in 
Bavaria  in  1889  and  1890  were  as  follows: 


Age. 

Per  cent 

1 

1.5 

2-5 

5.4 

6-10 

6.6 

11-15 

7.2 

Age. 

Per  cent 

16-20 

11.4 

21-30 

22.2 

31-40 

19.3 

41-50 

12.6 

Epstein's  statistics  show  1.6  per  cent,  in  the  first  year,  12.5  per 
cent,  from  one  to  ten  years,  and  36J  per  cent,  from  ten  to  twenty  years. 
Strassmann  has  reported  cases  occurring  in  the  newborn. 

The  source  of  infection  in  the   newborn  and  young  infants  is   the 

Fig.  106. 


.  / 

/ 

II 

3 

Influenza  bacilli,    (a)  In  nasal  secretion;    (fc)  in  culture  with  bacillus  sputigenes  crassus; 
(c)  diphtheria  bacilli  for  comparison. 

mother,  j-et  nursing  babies  acquire  a  relative  immunity  through  the 
mother's  milk  as  is  observed  in  other  infectious  diseases. 

The  younger  the  cliild  the  more  marked  are  the  intestinal  symptoms, 
with  secondar}^  involvement  of  the  central  nervous  system,  which  gives 
it  more  the  nature  of  a  general  infection  or  intoxication.  The  symptoms 
on  the  part  of  the  respiratory  apparatus  become  more  pronounced  as 
the  cliild  grows  older. 

The  oni^et  of  the  disease  is  usually  sudden.  Cliildren  first  complain 
of  feehng  tired  and  indisposed  and  on  examination  appear  depressed 
and  listless,  complain  of  headache  and  show  some  fever.  There  is  a 
marked  distaste  for  food;  the  facial  expression  is  anxious;  cj'anosis  is 
frequent;  the  eyes  are  watery  and  sensitive  to  light;  and  herpes  cornete 
is  not  uncommon.  The  pul.^e  is  rapid  and  there  is  tenderness  of  the 
neck,  back  and  legs.  The  younger  the  child,  the  more  indistinct  is  the 
clinical  picture.  In  verj'  j'oung  children  the  disease  may  be  ushered  in 
by  vomiting  and  convulsions  and  other  symptoms  of  meningeal  irrita- 


452  THE   DISEASES   OF   CHILDREN 

tion,  also  intestinal  disturbance,  constipation,  diarrhoea,  coryza  and  a 
dry,  hacking  cough. 

The  above  brief  sketch  of  the  disease  must  be  described  in  detail. 

The  temperature  rises  rapidly  with  the  outset  of  the  disease  to 
40°- 41°  C.  (104°-105°  F.),  and  in  uncomplicated  cases  subsides  to 
normal  in  24  to  48  hours.  In  rare  cases  it  terminates  by  lysis  and 
may  resemble  the  typhoid  curve.  A  condition  of  collapse,  with  a 
temperature  of  30°  C.  (90°  F.)  or  less,  may  occur  in  infants  and 
children  with  meningeal  symptoms. 

Headache  generally  accompanies  the  fever  and  is  referred  by  older 
children  to  the  frontal  region,  less  often  to  the  temporal.  The  supra- 
orbital region  and  the  eyes  are  very  sensitive  to  pressure  due  to  cerebral 
congestion.     The  headache  is  very  severe. 

The  pulse  shows  nothing  typical  of  the  severity  of  the  disease.  It 
is  small,  compressible,  arrhythmic  and  of  varying  frequency. 

The  striking  exhaustion  and  subjective  lassitude  as  well  as  the  gen- 
eral hyperesthesia  are  symptoms  present  in  cliildren  equally  with  adults. 
The  tenderness  becomes  apparent  in  young  children  when  attempts  are 
made  to  Uft  or  move  them. 

Examination  of  the  buccal  cavity  and  pharynx  of  a  fresh  case 
shows  a  diffuse  and  characteristic  congestion  of  the  parts.  Soltmann 
in  1887  described  the  appearance  as  follows:  "Influenza  in  children 
does  not  begin  with  a  nasal  catarrh  but  with  a  retropharyngitis." 

As  a  number  of  diseases  in  children  are  characterized  by  a  hyper- 
emia of  this  region  it  is  necessary  to  describe  the  diagnostic  features. 
The  congestion  is  sharply  defined  from  the  unaffected  tissue.  Redness 
and  cyanosis  frequently  appear  in  hnes  along  the  surface  of  the  pharynx 
and  later  a  fibrinous  exudate  is  formed  which  strips  off.  The  entry 
into  the  system  occurs  through  the  pharynx. 

The  influenza  infection  may  first  show  itself  in  older  children  as  in 
adults,  with  a  coryza  having  a  scant,  thin  secretion.  A  dry  bronchitis 
with  a  paroxysmal  cough  may  follow  tliis.  The  younger  the  cliild  the 
less  pronounced  are  the  respiratory  symptoms. 

In  Schlossmann's  cases  under  three  years,  35  per  cent,  showed  no  or 
only  shght  broncho  or  pulmonary  involvement  at  the  beginning  of  the  dis- 
ease, while  in  cases  over  ten  years  of  age,  five-sixths  began  in  tliis  manner. 

The  coryza  is  accompanied  by  a  severe  congestion  of  the  nasal 
mucous  membrane  which  often  produces  nosebleed. 

The  cough  is  dry,  severe  and  racking  and  similar  to  that  in  the 
prodromal  stages  of  measles,  and  may  simulate  the  paroxysm  of  per- 
tussis. Some  authors  speak  of  a  "pseudopertussis"  in  the  course  of 
influenza.  This  irritative  cough  often  continues  during  the  period  of 
convalescence.  The  termination  of  the  congestive  catarrh  is  in  all  cases 
rather  late  and  the  discharge  may  finally  become  mucopurulent. 


INFLUENZA  453 

In  certain  epidemics  a  severe  and  dangerous  membranous  croup 
or  pseudocroup  has  been  reported.  Concetti  describes  this  as  the  "forma 
laryningea"  of  influenza. 

Complications  on  the  part  of  tlie  respiratory  system  unfortunately 
are  not  uncommon.  Bronchopneumonia  appears  in  its  characteristic 
way  in  older  children,  but  in  very  young  or  weak  infants  it  may  run  a 
latent  course  and  terminate  suddenly  in  a  fatal  outcome.  Lobar  pneu- 
monia occurs  rarely  but  in  an  outspoken  manner,  as  in  cliildhood.  The 
typical  influenza  pneumonia  so  thoroughly  described  by  Finkler  appears 
only  rarely  in  children.  Whether  the  pneumonia  is  a  specific  symptom 
of  the  disease  or  a  secondary  infection  on  a  fertile  and  predisposed 
ground  is  an  academic  question.  The  course  of  the  disease  as  well  as 
the  severe  systemic  toxaemia  stamps  tliis  as  a  very  serious  compUcation. 
Pleurisy  is  not  uncommon  and  Meunier  found  a  serous  effusion  in  ten 
out  of  eleven  cases. 

In  the  early  years  of  life  all  affections  of  the  respiratory  organs 
have  a  less  menacing  character  than  in  later  years.  The  danger  of  a 
secondary  tuberculous  infection  is  less  than  after  measles  or  pertussis. 

The  congested  condition  of  the  mucous  membranes  of  the  mouth 
and  throat  is  participated  in  by  the  conjunctivae.  Conjunctivitis  with 
photophobia  is  of  frequent  occurrence  and  reminds  one  of  the  prodromal 
symptoms  of  measles.  In  small  cliildren  the  inflammation  may  extend 
into  a  blepharitis  ciharis  and  a  resulting  spasm  of  thehds.  A  superfi- 
cial keratitis  and  herpes  cornese  have  been  observed.  Dakryocystitis 
may  follow  by  extension  from  the  nasal  mucous  membrane. 

The  ear  is  involved  by  a  direct  extension  of  the  inflammation  from 
the  pharynx  through  the  Eustachian  tube.  Nearly  every  child  suffer- 
ing from  influenza  complains  of  more  or  less  severe  earache.  Often  tliis 
is  simply  a  neuralgic  otalgia.  Inflammation  of  the  external  auditory 
canal  may  occur.  On  inspection  in  most  cases  a  dark  opaque  redness  of 
the  drum  membrane  is  seen,  wliile  in  the  severe  cases  either  small  or 
large  haemorrhages  may  be  detected.  This  haemorrhagic  middle  ear 
inflammation  may  develop  into  a  purulent  otitis  media.  When  tliis 
occurs  there  is  bulging  of  the  drum,  a  secondary  rise  in  temperature, 
and  severe  pain.  Relief  is  immediate  after  paracentesis,  and  influenza 
bacilU  may  be  detected  in  the  discharge.  Three-fourths  of  all  cases  are 
unilateral.  Mastoiditis  is  not  an  uncommon  termination.  Exceptionally 
there  may  be  severe  labyrinth  disease  (Habermann  had  a  case  in  a  two- 
year-old  child). 

Influenza  is  often  accompanied  by  a  severe  and  painful  tonsillitis 
which  may  become  follicular  and  covered  with  an  exudate. 

Gastro-intestinal  symptoms  were  not  formerly  regarded  as  a  part  of 
the  chnical  picture  of  influenza.  The  earlier  WTiters  regarded  influenza 
only  as  an  infectious  catarrh  of  the  upper  air-passages.    Now  the  diges- 


454  THE   DISEASES   OF   CHILDREN 

tive  apparatus  plays  an  important  part  in  young  children  and  infants 
and  in  many  cases  the  whole  course  of  the  disease  may  simulate  an 
acute  febrile  gastro-intestinal  catarrh. 

In  adults  we  find  loss  of  appetite,  vomiting  and  diarrhoea  as  pro- 
dromal symptoms.  The  younger  the  patient  the  more  marked  are 
these  symptoms.  Dyspepsia  of  varying  severity  is  present  in  nearly  all 
cases  in  children.  The  anorexia,  coated  tongue,  and  vomiting  are  sus- 
picious of  meningeal  irritation.  A  transient  constipation  followed  in  a 
few  days  by  diarrhoea  is  quite  common.  The  stools  are  fetid,  become 
more  frequent  and  fluid  and  together  with  the  other  symptoms,  marked 
prostration  is  produced.  These  intestinal  symptoms  may  become  so 
severe  as  to  simulate  typhoid  fever. 

The  intestinal  symptoms,  which  are  an  extension  of  the  inflamma- 
tory process,  prepare  these  parts  for  the  development  of  secondary  in- 
fections and  the  resulting  systemic  toxaemia. 

Enlargement  of  the  spleen  is  irregular  in  influenza  and  is  never 
marked. 

Albuminuria  occurs  in  from  six  to  ten  per  cent,  of  cliildren  suffer- 
ing from  influenza.  An  acute  nephritis  may  develop  similar  to  that  in 
scarlet  fever,  and  cases  of  the  acute  ha>inorrhagic  type  have  been 
reported.  A  case  of  secondary  pyelitis  following  influenza  has  been 
observed.  Anuria  may  occur  due  to  a  paralysis  of  the  bladder 
muscles  {m.  detrusor)  resulting  from  the  effect  of  the  toxin  on  the 
nervous  system. 

The  effect  of  the  toxaemia  is  especially  noticeable  in  the  involve- 
ment of  the  heart.  In  the  early  stages  of  the  disease  this  is  shown  by 
arrhythmia  and  tachycardia.  Later  on  the  heart  action  becomes  slower 
and  there  is  a  sUght  dilation  mth  indistinct  nmrmurs.  Tliis  may  result 
in  cyanosis  and  collapse.  The  toxaemia  shows  a  close  similarity  to  that 
of  diphtheria  in  its  effect  upon  the  innervation  of  the  heart  and  in  the 
parenchymatous  degeneration  of  the  heart  muscle. 

Tliis  relative  heart  weakness  must  be  borne  in  mind  for  a  long  time 
in  severe  cases.  True  and  even  severe  endocarditis  has  been  observed 
after  influenza. 

The  tliird  group  of  symptoms — nervous  influenza — are  found  in  all 
well-marked  cases.  The  severe  depression,  the  aching  bones,  the  weari- 
ness, the  pain  in  the  hmbs  and  muscles,  and  the  sensitiveness  of  the  spine 
are  all  included  in  the  term  nervous  or  rheumatoid  influenza.  Locahzed 
peripheral  nerve  tenderness  is  frequent;  but  true,  long-continued  neu- 
ralgias are  uncommon  in  cliildren. 

Of  much  greater  importance  is  the  involvement  of  the  brain  and  its 
membranes.  A  diagnosis  from  diseases  of  the  meninges  is  uncertain  in 
the  beginning  of  an  infectious  disease  with  Mgh  fever  as  in  influenza. 

Meningeal  irritation  or  meningismus  occurs  easier  and  much  oftener 


INFLUENZA  455 

in  young  children.     From  this  to  severe  inflammation  of  the  meninges 
we  find  all  degrees  of  involvement  diminisliing  in  intensity  with  age. 

These  symptoms  are  usually  due  to  the  toxin  except  in  the  fortu- 
nately rare  cases  where  there  is  an  exudative,  purulent,  or  parenchy- 
matous change  in  the  meninges.  Tliis  toxic  irritation  is  the  cause  of 
the  severe  headaches  with  the  exception  of  the  congestions,  the  eclamp- 
tic seizures  and  the  various  irritative  symptoms  (laryngospasmus)  so 
easily  developed  on  the  unripe  nervous  system  of  the  child.  In  young 
children  where  the  entire  family  is  affected,  a  stuporous  condition  is 
often  present  alternating  with  eclamptic  seizures.  A  slight  opacity  of 
the  meninges  was  the  only  post-mortem  finding  in  two  such  fatal  cases. 

True  meningitis  as  a  result  of  the  infection  is  either  due  to  the 
influenza  bacillus  or  is  secondary  to  a  secondary  infection  from  the 
middle  ear. 

The  bacillus  of  influenza  has  frequently  been  found  in  the  meninges 
and  has  been  obtained  by  lumbar  puncture  in  the  hving. 

Hemorrhagic  encephahtis  and  acute  poliomyehtis  have  been  ob- 
served after  influenza  and  severe  degenerative  changes  as  in  sclerosis 
have  been  described. 

Psychoses  of  a  more  or  less  severe  grade  are  occasionally  seen  during 
convalescence.  These  are  manifested  by  hallucinations,  cataleptic 
stupor,  etc.  The  mind  generally  returns  to  its  normal  condition  after 
strength  has  been  restored.  The  disease  may  be  ushered  in  by  mental 
confusion,  as  in  Ewald's  case  of  a  seven-year-old  boy  who  at  the  outset 
of  the  disease  went  to  the  railroad  station  and  boarded  a  train  instead 
of  going  to  school,  without  any  recollection  of  what  he  was  doing.  Many 
children  at  the  beginning  of  the  disease  fall  almost  in  a  condition  of 
lethargy  for  wliich  there  is  no  satisfactory  explanation.  Heubner  also 
found  it  during  convalescence.  Acute  dizziness  and  dehrium  when 
present  are  a  result  of  the  liigh  toxic  fever. 

In  most  cases  of  influenza  there  are  no  changes  in  the  sldn.  How- 
ever, it  is  not  at  all  uncommon  to  find  an  exanthem  in  the  course  of  tliis 
disease  upon  wliich  too  httle  emphasis  has  been  placed.  Generally  tliis 
is  a  small,  punctate,  confluent  erythema  with  itcliing  which  is  found  on 
the  chest,  abdomen  and  sides  of  the  extremities.  The  exanthem  in  the 
majority  of  cases  is  very  similar  to  that  of  scarlet  fever  and  this  may 
render  the  diagnosis  under  certain  conditions  exceedingly  difficult. 

Other  eruptions  may  appear  after  a  few  days  or  in  the  second  week. 
Filatow  and  others  have  described  an  eruption  similar  to  measles  and 
cases  resembUng  urticaria,  roseola,  and  purpura  have  been  described. 

It  is  doubtful  if  these  rashes  are  a  result  of  a  mixed  infection  with 
influenza.  In  a  large  number  of  exanthemic  infectious  diseases  the  in- 
fluenza bacillus  has  been  found  in  the  blood  and  different  organs.  A 
mixed  infection  with  diphtheria  has  been  reported  and  Jochmann  found 


456  THE   DISEASES   OF   CHILDREN 

the  influenza  bacillus  in  a  case  of  pertussis.  Many  authors  have  found 
the  influenza  bacillus  to  be  the  cause  of  a  pneumonia  occurring  in  the 
course  of  other  diseases. 

The  lymphatic  system  plays  a  small  part  in  influenza. 

An  uncomphcated  case  of  influenza  is  generally  only  of  a  few  days 
duration  but  it  may  be  prolonged  several  weeks.  The  various  complica- 
tions make  the  course  of  the  disea.sc  very  uncertain.  Tliis  is  especially 
true  when  the  lungs  are  involved  and  a  chronic  congestion  results  which 
is  very  slow  in  disappearing.  Filatow  and  others  have  described  a  pro- 
tracted form  of  influenza  wliich  may  persist  for  months  without  any 
special  catarrhal  manifestations,  but  showing  a  mild  degree  of  fever, 
headache  and  weakness.  The  possibihty  of  tuberculosis  must  not  be 
overlooked. 

The  prognosis  of  uncomphcated  influenza  in  children  is  favorable 
even  when  the  lungs  are  involved  but  it  should  be  guarded  when  the 
heart  is  affected.  In  general  the  prognosis  depends  upon  the  sympto- 
matology of  the  individual  case.  An  influenza  grafted  on  an  existing 
tuberculosis  is  very  unfavorable. 

The  diagnosis  of  influenza  in  children  is  not  always  simple  when 
there  is  no  epidemic,  but  when  whole  districts  are  affected  it  is  a  very 
easy  matter.  At  such  times  the  possibihty  of  other  diseases  with  symp- 
toms similar  to  influenza  should  be  borne  in  mind.  After  the  exclusion 
of  other  diseases,  the  symptom-complex  on  the.  part  of  the  various 
organs,  the  height  of  the  fever  and  extreme  weakness,  the  typical 
appearance  of  the  mouth  and  throat  all  tend  to  estabhsh  the  diagnosis. 

Unfortunately  the  bacteriological  diagnosis  is  seldom  available 
for  practical  purposes.  It  is  difficult  to  make  the  diagnosis  when  the 
symptoms  described  as  meningismus  are  present.  Have  we  to  deal 
with  a  beginning  influenza,  or  some  form  of  true  meningitis?  In  influ- 
enza we  may  find  some  retraction  of  the  head  with  tenderness  on  motion 
and  irregular  pulse  and  respiration.  Rapidity  and  arrhythmia  of  the 
pulse  are  characteristic  of  influenza  when  it  is  present.  Lumbar  punc- 
ture in  these  cases  is  of  the  greatest  diagnostic  importance. 

In  the  differential  diagnosis  several  possibihties  must  be  borne  in 

mind.    Influenza  with  sore  throat  and  a  scarlatiform  rash  may  simulate 

•  scarlet  fever  but  the  course  of  the  fever  and  rapid  disappearance  of  the 

rash  will  suflftce  in  most  cases  to  clear  the  diagnosis.     Desquamation 

and  infection  of  the  lymphatic  nodes  never  occur  in  influenza. 

In  measles  there  is  a  longer  prodromal  period,  the  rash  reaches  its 
height  in  three  to  four  days,  the  presence  of  Kophk's  spots,  the  severe 
conjunctivitis,  the  sneezing,  etc.,  all  speak  against  influenza.  Typhoid 
■  is  recognized  by  the  enlarged  spleen,  roseola,  and  diazo  and  Widal 
reactions.  The  possibihty  of  miliary  tuberculosis  may  come  in  ques- 
tion for  a  few  days. 


INFLUENZA  457 

The  pathology  of  influenza  has  nothing  distinctive.  There  may  be 
the  characteristic  appearance  of  pulmonary  comphcations  with  small 
areas  of  consolidation  wliich,  deeply  congested  or  htemorrhagic,  are 
about  the  yellow  stained  bronchi  and  separated  by  rather  firm  round- 
cell  infiltration  of  the  connective  tissue.  Abscess  and  necrosis  of  the 
lung  are  not  uncommon. 

There  are  no  distinctive  changes  in  the  spleen,  heart,  fiver  and 
kidneys  except  that  the  influenza  bacillus  can  be  cultivated  from  them. 
The  other  changes  such  as  inflammation  of  the  gastric  nmcous  membrane 
are  purely  symptomatic. 

The  prophylaxis  depends  upon  avoidance  of  cold  and  exposure  and 
removal  from  a  locahty  or  school  where  influenza  is  epidemic.  CMldren 
at  such  times  should  be  kept  away  from  crowds,  meetings,  and  pubfic 
conveyances. 

The  treatment  is  in  a  great  measure  purely  symptomatic.  The 
management  of  comphcations  such  as  pneumonia,  otitis,  meningitis, 
etc.,  is  described  in  detail  in  other  chapters. 

The  cliild  must  be  kept  in  bed.  The  diet  should  be  generous,  not  a 
starvation  diet,  but  one  rich  in  carbohydrates,  poor  in  proteids  and  pref- 
erably fluid  at  first.  Nursing  infants  should  not  be  taken  away  from 
the  breast.  Older  cliildren  take  with  benefit  copious  amounts  of  water, 
lemonade,  fruit  juices,  etc.  Hydrotherapy  is  of  great  advantage  espe- 
cially in  young  cliildren.  Cold  packs  with  lukewarm  baths  and  rubbing 
are  helpful.  Heubner  advises  placing  the  cliild  in  a  warm  bath  and  pour- 
ing cold  water  on  the  chest  to  overcome  the  intense  stupor  in  some 
cases.     Cold  baths  should  be  employed  to  reduce  the  fever. 

There  are  no  specific  drugs  in  influenza.  The  saficylate  of  quinine 
in  small  doses  is  of  benefit.  Aspirin  and  the  benzoate  of  soda  are  useful 
in  older  children.  The  benzoate  of  soda  is  both  antiseptic  and  antipy- 
retic and  has  no  unfavorable  after-effects.  Inhalation  of  the  tincture  of 
benzoin  is  very  sootliing  to  the  inflamed  mucous  membrane.  For  the 
bronchitis  and  diarrhoea  small  doses  of  tinctura  opii  benzoica  are  useful. 
Antineuralgic  drugs  may  be  employed  to  ease  the  earache. 


WHOOPING-COUGH 

BY 

Dr.  R.  NEURATH,  of  Vienna 

translated  by 
Dr.  frank  X.  WALLS,  Chicago,  III. 


Historically,  whooping-cough  may  be  regarded  as  a  recent  dis- 
ease. The  first  description  of  a  whooping-cough  epidemic  we  owe  to 
DeBaillou  (1578)  and  in  the  seventeenth  century  Willis,  Sydenham, 
and  others  recognized  the  disease  and  differentiated  it  from  those  symp- 
tomatologically  related  to  it.  Whooping-cough  has  since  become  an 
affection  common  to  all  latitudes,  with  a  tendency  to  assume  every  year 
in  unequal  waves  an  epidemic  character.  The  frequency  of  epidemics 
and  the  opportunity  that  has  existed  daily  for  decades  and  centuries, 
to  observe  the  disease;  its  symptoms,  recognizable  even  by  a  layman, 
and  the  fact  that  for  the  purpose  of  estabhsliing  a  diagnosis  even  the 
recent  finer  methods  of  examination  may  be  dispensed  with, — all  these 
factors  have  contributed  towards  advancing  the  symptomatology  and 
diagnosis  of  whooping-cough  (Biermer,  Barthez  anil  Rilliet,  Hagenbach, 
Sticker),  whereas  its  pathogenesis  and  etiology  are  still  under  discussion, 
in  spite  of  laborious  investigations  made  with  modern  equipment. 

Whooping-cough  is  a  contagious  infectious  disease.  The  primary 
symptoms  invoh-ing  the  respiratory  tract,  the  transmission  of  the  dis- 
ease through  the  sputa,  and  the  usually  afebrile  course  in  uncomplicated 
cases  might  be  regarded  as  criteria  of  a  local  infectious  disease.  But 
the  occurrence  of  an  initial  fever  wave,  which  close  observation  reveals, 
and  which  does  not  run  parallel  with  the  respiratory  signs,  an  early 
inflammatory  leucocytosis,-  the  similarity  of  the  initial  symptoms  to 
those  of  other  general  affections,  the  permanent  immunity  almost  al- 
ways acquired  by  those  who  have  had  an  attack  of  pertus-sis,  the  course 
and  mechanism  of  the  attacks  of  coughing  differing  from  other  condi- 
tions of  local  irritation  of  the  respiratory  mucous  membrane,  and 
finally  certain  comphcations  that  probably  can  be  accounted  for  by  the 
action  of  toxins,  justify  one  in  considering  whooping-cough  as  a  general 
infectious  disease  whose  portal  of  entry  and  principal  symptoms  are 
found  in  the  respiratory  tract. 

The  transmission  of  the  disease  from  the  sick  to  the  well  is  usually 
direct  and  immediate,  in  a  great  majority  of  cases,  through  the  sputum 
scattered  by  coughing.     A  mediate,  indirect  infection  through  sputum 

458 


WHOOPING-COUGH  459 

adhering  to  clothes,  handkerchiefs,  playthings,  benches,  etc.,  is  very 
rarely  met  with.  The  virulency  of  the  sputa,  that  is  to  say,  the  dura- 
bihty  of  the  exciting  cause,  lasts  only  a  short  time. 

The  susceptibility  to  whooping-cough  varies  especially  with  the  age 
of  individuals  exposed  to  the  danger  of  infection.  Statistics  proving  the 
enormous  prevalence  of  pertussis  in  children  justify  the  designation  of 
whooping-cough  as  a  chsease  of  cliildhood.  Those  who  have  reached  the 
age  of  puberty  are  far  less  susceptible  to  pertussis  than  to  other  acute 
infectious  diseases  occurring  chiefly  in  childhood. 

A  collection  of  the  cases  of  whooping-cough  reported  in  the  city  of 
Vienna,  in  1899,  1900  and  1901  as  published  in  the  statistical  yearbooks, 
shows: 

In  the  first  year  of  life 1242  cases. 

From  the  second  to  the  fifth 3139  cases. 

From  the  sixth  to  the  tenth 1926  cases. 

From  the  eleventh  to  the  fifteenth 135  cases. 

From  the  sixteenth  to  the  twentieth 12  cases. 

From  the  twenty-first  to  the  twenty-fifth 2  cases. 

Beyond  the  twenty-sixth  year 13  cases. 

The  age  before  compulsory  school  attendance  is  most  affected. 
The  susceptibility  shared  by  all  cliildren  makes  every  case  imported 
into  the  family  from  the  school,  the  nursery,  the  kindergarten  or  the 
pubhc  playground  a  starting-point  for  a  house  epidemic  likely  to  attack 
the  youngest  of  children.  The  first  year  of  hfe,  as  seen  from  the  above 
statistics,  is  less  often  involved  than  the  following,  but  nevertheless  it 
is  to  a  considerable  extent  even  more  than  by  measles.  Whether  this  is 
due  to  a  certain  lowered  susceptibility  or  to  the  less  frequent  contact 
with  other  cliildren,  especially  in  cases  of  first-born,  cannot  be  certainly 
decided.  But  that  the  first  few  days  or  weeks  of  infantile  hfe  do  not 
mean  immunity  from  whooping-cough,  may  be  proven  by  many  closely 
observed  cases.  Bouchut  reports  a  case  in  which  a  newborn  cliild,  in- 
fected on  the  second  day,  began  to  cough  on  the  fourth  day,  and  on  the 
eighth  day  had  pronounced  paroxysms  of  whooping-cough.  RilHet  and 
Barthez  reported  a  case  of  pertussis  in  a  newborn  child  whose  mother 
had  whooping-cough.  Watson  observed  whooping-cough  on  the  first 
day  of  hfe,  and  I  know  of  a  case  in  a  clrild  fourteen  days  old.  Porak  and 
Durante,  during  a  local  epidemic  at  the  Paris  Maternite  noticed  a  lesser 
disposition  to  whooping-cough  in  the  prematurely  born  (10  cases  out 
of  44),  but  a  greater  susceptibihty  in  those  born  at  term,  mostly  fed  by 
different  wet-nurses  (10  cases  out  of  14),  all  these  infants  being  less  than 
one  year  old.  At  the  other  extreme  of  hfe,  cases  of  whooping-cough  are 
known  to  have  occurred  during  old  age. 

All  observations  made  on  an  extensive  scale  show  that  the  female 
sex  is  the  more  largelv  involved.     From  1899  to  1901,  6666   girls   as 


460  THE   DISEASES   OF   CHILDREN 

against  5127  boys  were  officially  recorded  in  Vienna  as  having  been 
affected  with  whooping-cough. 

Wliile  in  large  cities  pertussis  never  disappears  entirely  and  the 
preponderating  involvement  of  the  first  years  of  Hfe  in  the  morbidity 
of  whooping-cough  may  be  regarded  as  the  expression  of  a  general  sus- 
ceptibihty  of  man,  to  which  even  the  very  youngest  are  subject,  remote 
countries,  cut  off  from  the  outside  world  (e.^f.  the  Faroe  Islands),  are 
apt  to  become  rapidly  infected  from  a  single  exposure  and  to  suffer  a 
general  dissemination,  of  the  disease.  The  immunity  acquired  by  a 
large  percentage  of  persons  who  early  in  hfe  have  had  an  attack,  as 
well  as  a  certain  reduction  of  their  susceptibility,  affords  a  protection 
to  adults. 

Attempts  made  in  various  quarters  from  observations  extending 
over  decades  to  determine  a  certain  periodicity  for  the  epidemics  of 
whooping-cough  have  not  led  to  any  satisfactory  conclusions.  Neither 
season  nor  weather  nor  other  conditions  such  as'  the  increased  contact 
of  cliildren  at  the  beginning  of  school,  appear  to  occasion  an  epjdemic- 
hke  expansion  of  the  morbidity  curves. 

Figures  arranged  according  to  months  sho-uang  the  number  of 
cases  of  whooping-cough  officially  recorded  in  Vienna  during  five  years 
(1898  to  1902)  exMbit  the  highest  number  for  March  (1332)  and  April 
(1243),  the  lowest  for  October  (555).  Other  observers,  however,  report 
differently. 

It  seems  that  the  raw  periods  of  the  year  more  frequently  cause  an 
increase  in  the  morbidity  figures  and  a  protracted  duration  of  single  epi- 
demics, corresponding  to  the  deleterious  influence  exercised  by  inclement 
weather  in  general  on  affections  of  the  respiratory  tract.  The  alterna- 
tion of  measles  and  whooping-cough  epidemics,  sometimes  observed  but 
often  overestimated  as  to  its  frequency,  may  possibly  be  accounted  for 
in  a  similar  manner.  In  both  infectious  diseases  a  distinct  involvement 
of  the  respiratory  tract  is  a  prominent  feature;  in  both,  the  portal  of 
entrance  of  the  pathogenic  factor  is  presumed  to  be  in  the  mucous  mem- 
brane of  the  respiratory  tract.  Thus  a  catarrh  accompan3ang  one  of 
these  diseases  might  easily  facihtate  an  infection  with  the  other. 

Since  the  disease  has  been  completely  described  symptomatically, 
attempts  based  on  anatomical  and  chnical  experience  have  been  made 
to  investigate  the  etiology  and  pathogenesis  of  whooping-cough.  The 
favorable  prognosis  of  uncomphcated  cases  prevents  obtaining  post- 
mortem findings  undoubtedly  underlying  the  basal  disease.  After  death, 
we  find  almost  always  lobular-pneumonia  and  tuberculous  alterations  of 
the  lungs  and  lymph-nodes,  together  with  evidences  of  pulmonary  em- 
physema, dilatation  and  hypertrophy  of  the  heart,  changes  in  the  cen- 
tral nervous  system  (see  nervous  comphcations)  capillary  haemorrhages, 
etc.,  according  to  the  chnical  comphcations.     The  ever-present  catarrh 


WHOOPING-COUGH  461 

of  the  respiratory  mucous  membrane  and  its  localization  as  may  be  deter- 
mined laryngoscopically  during  life  should  support  the  theory  that 
whooping-cough  is  a  specific  catarrh  causing  because  of  its  locahzation, 
a  spasmodic  cough.  However,  laryngoscopic  findings,  credited  almost 
exclusively  to  older  investigators,  differ  quite  considerably,  both  wath 
regard  to  the  intensity  and  especially  the  localization  of  the  alterations. 
But  it  seems  that  the  rima  glottidis  posterior  is  the  point  of  irritation 
at  which  the  tenacious  httle  lumps  of  sputum,  rich  in  mucin,  driven 
up  from  the  deeper  bronchial  branches  by  the  movements  of  cihated 
epithehum,  excite  the  paroxysm. 

Against  the  conception  of  whooping-cough  as  a  specific  laryngo- 
tracheitis,  the  follo\\'ing  facts  have  been  justly  brought  forward:  the 
rhythmic  process  of  each  paroxysm  and  of  the  whole  affection  differing 
from  such  catarrhs,  the  influence  exercised  on  the  symptoms  by  the 
state  of  mind,  the  convulsion  phenomena  during  and  between  the 
individual  attacks,  and  finally  the  frequent  inactivity  of  narcotics, 
especially  in  the  catarrhal  stage.  These  facts  were  more  apt  to  cause 
whooping-cough  to  be  numbered  among  the  functional  nervous  diseases. 
But  its  acute  onset  and  undoubted  contagiousness  as  shown  by  every- 
day observations  make  the  inclusion  of  pertussis  among  the  contagious 
infectious  diseases  the  only  justifiable  one. 

The  transtnission  of  whooping-cough  from  an  infected  to  a  healthy 
individual  naturally  led  to  the  assumption  that  the  pathogenic  \'irus 
was  to  be  looked  for  in  the  sputum  and  the  mechanism  of  the  process  in 
the  infection  by  aspiration  of  httle  drops  of  infectious  expectorated 
matter.  There  are  two  possibihties  to  consider:  whooping-cough  may 
be  regarded  either  as  a  local  infectious  chsease  of  the  respiratory  tract, 
the  irritant  being  produced  in  the  larynx  or  trachea,  and  (contrarj'  to 
most  other  infectious  local  diseases)  by  reaction  of  their  toxic  products 
established  a  permanent  immunity  in  those  who  had  once  been  affected; 
or  it  may  be  considered  as  a  general  infectious  disease  winch,  starting 
primarily  from  the  mucous  membrane  of  the  respiratory  tract,  produces 
the  chnical  symptoms  through  toxic  products.  As  already  stated,  a 
number  of  weighty  facts  speak  for  its  acceptance  as  a  general  infectious 
disease. 

The  exciting  factors  of  whooping-cough  have  been  searched  for 
throughout  many  decades,  since  Linnaeus,  and  the  results  obtained  time 
and  again,  seemingly  successful,  have  brought  forward  organisms  of  the 
greatest  biological  variety.  Deichler,  Kurloff,  and  Behla  thought  proto- 
zoa were  the  cause  of  pertussis,  while  Moncorvo  and  Silva-Aranja, 
Broadbent,  Haushalter,  Mircoh,  Ritter,  and  others  regarded  cocci  as  the 
pathogenic  factors;  Burger,  Afanassiew,  Szemetschenko,  Wendt,  and 
Genser  considered  bacilli  as  the  causative  agents.  Quite  recently  Cza- 
plewski  and  Hensel  on  one  hand,  Jochmann  and  Krause  on  the  other, 


462  THE   DISEASES   OF   CHILDREN 

and  finally  Manicatide  have  described  certain  bacilli  which  may  bring 
us  nearer  to  a  bacteriological  knowledge  of  whooping-cougli. 

Czaplewski  and  Ilcnsel  found  in  the  expectorations,  after  staining 
with  carbol-glycerin-fuchsin  and  treating  the  preparations  with  1  per 
cent,  acetic  acid,  many  small  short  bacilh  wth  rounded  corners,  about 
the  size  of  an  influenza  bacillus,  but  differing  from  the  latter  in  that 
they  grow  on  ordinary  culture  media.  The  bacilli  are  two  to  three  times 
as  long  as  broad.  If  deUcately  prepared,  polar  staining  is  shown;  while 
if  strongly  stained,  total  staining  is  accompHshed.  Experiments  on 
animals  proved  negative,  but  their  regular  occurrence,  according  to 
the  authors,  is  suggestive  of  specificity.  Cavasse,  Wagner,  von  Zusch, 
Koplik,  Arnheim,  and  Reyher  were  able  on  the  whole,  to  confirm  the 
findings  (see  Fig.  107). 

Jochmann  and  Krause,  in  their  investigations  made  at  the  Ham- 
burg-Eppendorf  hospital,  found  in  whooping-cough  sputa  in  a  majority 
of  cases  tiny,  influenza-Uke  bacilh  of  morphological  identity.  However, 
these  they  say,  do  not  belong  to  one  species,  but  represent  three  different 
ones  distinguished  from  each  other  biologically,  for  example  by  their 
behavior  towards  Gram's  method  of  staining.  The  influenza-Hke  bacillus 
found  by  Czaplewsld  and  Hensel  was  detected  by  Jochmann  and  Krause 
in  only  four  cases.  The  latter  claim  as  the  cause  of  whooping-cough  an 
influenza  like  Gram-negative  bacillus  (Bacillus  pertussis  Eppendorf), 
gro\\ing  only  on  culture  media  containing  haemoglobin.  They  found 
this  organism  in  eighteen  cases. 

A  third  type  of  bacillus  resembUng  those  of  influenza,  growing  in  all 
ordinary  culture  media  and  being  Gram-positive  (bacillus  z),  was  found 
by  Manicatide.  Its  specificity  he  claims  to  have  estabhshed  by  sero- 
therapeutic  experiments,  immunizing  three  sheep  and  two  horses  and, 
by  injection  of  the  serum,  effecting  a  cure  or  at  least  an  improvement 
in  positive  whooping-cough  cases. 

The  bacteriological  investigations  carried  on  during  the  last  few 
years  have  shed  much  light  on  the  bacteriology  of  whooping-cough,  an 
oval  short  bacillus  resembling  the  influenza  bacillus  being  generally  re- 
garded as  the  specific  pathogenic  factor.  The  biological  details  are  still 
matters  of  contention,  but  control  experiments  (made  by  Reyher)  are 
strongly  suggestive  of  the  specificity  of  Czaplewski 's  polarbacterium. 

Concerning  the  period  of  incubation,  of  whooping-cough  hardly  any- 
thing positive  is  known.  According  to  the  experience  of  most  observers 
it  varies  between  three  and  fifteen  days.  If  three  weeks  after  exposure 
to  infection  the  disease  does  not  develop,  the  cliild  in  question  may  be 
regarded  as  not  infected. 

With  regard  to  the  contagiousness  of  whooping-cough  in  the  various 
stages  of  the  process  we  find,  especially  among  French  investigators, 
divergent  statements.     Extensive  observations  and  the  importance  of 


WHOOPING-COUGH  463 

greater  prophylactic  safety  make  it  more  advisable  to  regard  whoop- 
ing-cough as  contagious  during  its  whole  course — that  is,  to  keep  a  child 
suffering  from  whooping-cough  isolated  from  healthy  cliildren  so  long 
as  there  is  cough  with  expectoration.  Recovery  from  whooping-cough 
almost  always  insures  immunity  for  the  remainder  of  life,  according  to 
an  overwhelming  array  of  observations.  Analogous  to  those  infectious 
diseases  which  have  been  etiologically  more  definitely  investigated,  tliis 
fact  of  immunity  in  whooping-cough  may  hkewise  be  accounted  for  by 
reaction  products  evolved  by  specific  bacterial  metabohsm.  Against 
such  purely  theoretic  considerations  speaks  the  great  uncertainty  of 
our  present  etiologic  investigation,  in  that  cliildren  nourished  from 
their  mother's  breast,  even  during  their  infancy,  are  not  uncommonly 
affected,  wlien  we  might  expect  a  certain  lessened  susceptibihty  of  the 
infant  on  account  of  the  transference 

Fig.  107. 

through    the    mother's    milk    of   such  iv 

antitoxin   from   the   actively  immune  J^^  rr-^-^    X 

mother,  who  has  had  the  disease.  /a^w'        *  - /■' 


J 

■'~ 

/h 

-  -    ,■- 

It  is  possible  that  a  cliild  may  ( 
become  affected  with  pertussis  a 
second  time — an  interval  of  complete 
health  extending  over  years  preclud-  (^ 
ing  a  relapse  of  the  first  affection — but 
in  such  an  event,  the  course  of  the 
disease  is  rapid  and  Ught. 

Symptomatology. — For  a  descrip- 
tion of  the  normal  process  of  whoop-  =    .      •     u     ■  u   =.  ■   j    •  u 

'  ^  Sputum  in  whooping-cough.    Stained  with 

ing-cough  the  hitherto  usual  distinction        i  ?«■■  ■=™'-  ^"^"'^  ="^'"1  ""d  diluted  carboi- 

glycerin. 

of  the  several  stages  may  be  retained 

with  advantage,  although  a  strict  separation  of  the  phases  iii  a  given 
case  is  frustrated  by  the  frequent  exacerbations  and  comphcations. 
We  may  retain  the  customary  di\dsion  into  three  stages,  with  a  shghtiy 
characterized  prelude  of  prodromal  symptoms,  such  as  lassitude,  head- 
ache and  disturbed  sleep. 

The  first  or  catarrhal  stage  sets  in  with  symptoms  of  an  acute 
catarrhal  affection  of  the  respiratory  organs,  sneezing,  conjunctival  irri- 
tation, more  or  less  severe  cough,  and  sometimes  with  shght  fever.  Now 
and  then  a  shght,  enanthema-Uke  injection  of  the  palate  and  palatal 
arches  is  noticed.  In  this  stage,  as  a  rule,  every  auscultatory  sign  of 
bronchitis  is  wanting.  In  younger  children,  especially  in  such  as  in- 
cUne  to  false  croup,  an  attack  of  acute  larjmgitis  may  abruptly  open  the 
scene  and  in  the  next  few  days  subside  to  the  usual  catarrhal  symp- 
toms. For  one  or  two  weeks,  the  temperature  being  normal  and  the 
general  condition  relatively  good,  the  cough  increases,  becomes  spas- 
modic, choking,  more  frequent  by  night  than  by  day,  and  gradually 


464  THE   DISEASES   OF   CHILDREN 

assumes  the  typical  character  of  the  whooping-cough  paroxysms.  Thus 
the  catarrhal  phase  quite  gradually  glides  into  the  convulsive  stage. 
Tills  first  period  of  whooping-cough  lasts  one  to  two  weeks,  usually  from 
seven  to  ten  days. 

While  in  the  first  phase  of  pertussis,  especially  during  the  first  days, 
we  find  in  the  symptoms  notliing  characteristic  of  the  disease  and  we 
are  left  in  doubt  as  to  the  diagnosis;  so  that  in  the  end  only  a  positive 
history  of  exposure  or  the  inefficiency  of  narcotics  direct  our  suspicion 
to  whooping-cough.  The  pecuHarities  of  a  single  paroxysm  and  the  ob- 
jective results  of  an  examination  in  the  second  or  convulsive  stage,  will 
secure  a  positive  diagnosis.  The  attacks  occur  either  spontaneously  or 
may  be  aroused  by  a  variety  of  causes,  such  as  emotion  (anger,  laugliing, 
weeping),  reflex  acts  (singultus,  sneezing),  swallowing  of  sohd  morsels, 
more  copious  meals,  a  draught  of  cold  water,  an  air  current,  \'isual  and 
auditory  impressions  (glaring  fight,  shrill  sounds),  seeing  or  hearing  of 
a  whooping-cough  paroxysm  in  another  child.  Spontaneously  the 
paroxysms  occur  also  during  sleep,  by  night  even  more  frequently  than 
by  day. 

Older  children,  able  to  relate  their  sensations,  describe  aura-fike 
prodromes  introducing  the  paroxysm,  as  tickhng  or  scratcliing  in  the 
throat,  choking,  eructation,  suffocating  distress,  pressure  behind  the 
sternum,  intense  anxiety.  These  sensations  impel  the  children  to  run 
to  the  mother,  take  hold  of  sohd  objects  for  a  support,  or  rise  rapidly 
from  the  bed.  After  a  short  pause  in  breatliing  and  with  some  swallow- 
ing or  choking  movements  a  deep  inspiration  follows;  and  then  begins 
without  inspiration  and  rapidly  repeated,  a  series  of  staccato-hke,  short 
and  loud  forcible  expiratory  efforts  which  through  the  auxiliary  respi- 
ratory muscles,  convulse  the  entire  body.  The  face  and  the  visible 
mucous  membranes,  meanwhile,  become  Uvid,  the  eyeballs  protrude,  a 
watery  discharge  oozes  from  the  eyes,  and  the  tongue  becomes  puffed 
up,  deeply  cyanotic,  with  scaphoid  vaulting,  and  during  the  expiratory 
efforts  projects  from  the  mouth.  Finally,  after  a  succession  of  such  short 
coughing  efforts  follows  a  deep,  crowing  inspiration,  the  rima  glottis 
being  narrowed,  the  intercostal  spaces  and  clavicular  fossa*  contracted, 
and  the  hyoid  bone  drawn  upwards.  Immediately  after  there  is  a  repe- 
tition of  the  spasmodic  expiratory  efforts,  uninterrupted  by  inspiration 
continuing  until  again  succeeded  by  a  long-drawn,  crowing  inspiration. 
Finally,  at  the  close  of  the  attack  -ivith  choking  and  vomiting,  a  scant, 
tenacious  glairy  mucus  is  expelled.  Previously  there  may  occur  an 
apparent  cessation  of  the  paroxysm,  a  brief  pause  marked  by  a  few 
quiet  respirations,  but  very  soon  followed  by  a  new  attack  of  staccato 
efforts,  by  a  repetition  of  the  whole  disease  aspect, — a  "reprise.'' 
(By  many  the  whooping,  crowing  breathing  is  erroneously  designated 
as  reprise.)      During   the   coughing   fits,   not  infrequently  involuntary 


^VHOOPIXG-COUGH  465 

expulsion  of  feces  and  urine  may  occur,  due  to  the  vigorous  force  of 
the  abdominal  pressvu'e. 

The  paroxysm,  whose  duration  (usually  2  to  5  minutes)  is  apt  to  be 
overestimated  bj''  the  awe-stricken,  helpless  layman,  having  terminated 
with  the  evacuation  of  tenacious  mucus  or  ^^^thout  it,  stronger  and 
hardier  children  may  be  seen  quietly  resuming  their  interrupted  play, 
their  meal,  or  even  speech,  without  any  sign  of  distress.  Others,  on  the 
contrary,  after  the  attack  exliibit  lassitude,  sweating,  and  acceleration 
of  the  pulse  and  respiration.  Generally,  if  there  are  no  comphcations, 
the  subjective  condition  of  the  cliildren  is  very  good,  far  better  than  in 
the  catarrhal  stage,  the  cough-free  periods  affording  them  the  quiet  of 
full  health.  Subjective  distress  during  the  pauses,  great  exhaustion  in 
spite  of  a  small  number  of  paroxysms,  and  especially  elevations  of  tem- 
perature are  always  the  first  symptoms  of  incipient  complications. 

The  number  of  attacks  depends  above  all  on  the  gravity  of  the 
affection  and  on  constitutional  factors,  as  well  as  on  cHmatic  and  general 
hygienic  conditions.  In  \Tgorous  children  and  in  uncomplicated  cases 
the  paroxysms  may  be  only  ten  or  even  less  in  24  hours,  while  on  the 
other  hand  there  are  children  who  have  to  endure  forty,  or  even  fifty 
and  sixty  spasmodic  attacks.  The  number  of  these  paroxysms  may  be 
easily  controlled  and  the  attending  nurse  should  be  instructed  to  mark 
each  attack  by  a  sign  on  the  history  sheet. 

The  convulsive  stage  in  its  course  is  characterized  by  three  phases 
— an  increase,  an  acme,  and  a  decrease  of  the  wave.  The  decrease  man- 
ifests itself  rather  in  a  change  in  the  severity  of  the  single  paroxysm 
than  in  a  diminution  of  the  number  of  attacks.  The  latter  run  their 
course  entailing  less  effort  on  the  part  of  the  patient;  the  inspirations 
being  more  marked  and  effective,  the  spasmodic  cough  more  rapidly 
draws  to  an  end,  and  an  earher  expulsion  of  the  sputum  with  choking 
and  vomiting  occurs.  Without  sharp  dehmitation  and  gradually,  the 
course  of  the  disease  begins  to  improve,  reaching  the  stage  of  dechne 
which  is  a  second  catarrhal  phase. 

The  duration  of  the  convulsive  stage  cannot  be  easily  fixed,  fre- 
quent exacerbations,  even  in  uncomplicated  cases,  being  apt  to  modify 
the  course.  Thus,  we  can  determine  the  normal  duration  only  in  those 
cases  which  run  a  rapid  course.  Two  to  three  weeks  may  generally  be 
set  down  as  the  shortest  duration  of  the  second  stage.  But  right  here 
we  have  to  emphasize  that  damage  due  to  careless  conduct,  chmatic 
conditions,  severity  of  the  infection,  constitutional  and  especially  com- 
plicating diseases  may  prolong  the  con\Tilsive  stage  for  weeks  and 
months. 

The  last  stage  (stage  of  decline)  marks  the  disappearance  of  the 
symptoms.  The  attacks  occur  more  rarely,  their  course  is  milder  and 
more  rapid,  a  few  coughing  efforts  constitute  the  whole  paroxysm  sug- 

11—30 


466 


THE   DISEASES   OF   CHILDREN 


gestive  of  a  severe  productive  broncliial  catarrh,  with  inucus  or  muco- 
purulent expectoration.  The  daily  number  of  paroxj'sms  decreases, 
dropping  to  3,  2,  1,  until  finally  there  occurs  only  one  every  few  days. 
During  this  phase  of  whooping-cough  the  children  feel  cjuite  well.  But 
even  in  tliis  period  adverse  conditions  may  cause  a  relapse  into  the 

Fig.  108. 


Month    Jan. 


Day 


February 


March 


:  -?•  ic  '^  t-  GC  C--  o  - 


Cl  CO  -)^  LO  -^  I  -  oc  ffl  C  - 


:  r~  x  — '  ?i « -^  o  w  t..  X  ff.  o  ^  CI 


,3.5 
30 
25 
20 
1.5 
10 


iiiiiiiiiiiimiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiii 
iisiiiiiiiiiiiiiiiiiRiiiiiiiiimigiiiiiimiiiiiiimi 

IIIIISiM&liSSISIiSiSlfiilimilllliPKIIIIIIIIIIIIIIIIIII 
l|llllllillllllilllllllB!EilMK!lliglEi^lllllllllllllll 

iiiiiiiiiiiiiiiiiiiiaiiiissiiiiisiiiiiiiiimi 
miiiiiiiiiiiiiiiiiiiiiiiiiiiiiiiniiiKSiii 


Ill 


Whooping-cough  of  moderate  severity.     Marie  W.,  seven  years  old. 

paroxysmal  stage  and  revive  for  days  and  vyeeks,  all  the  symptoms  of 
the  convulsive  attacks.  In  light  cases  this  last  phase  of  dechne  lasts 
from  one  to  two  weeks  (Fig.   108). 

In  the  pauses  that  are  free  from  paroxysms,  especially  during  the 
convulsive  stage,  we  meet  with  symptoms  of  great    diagnostic  value. 
Fig.  109.  During  a  paroxysm  there   oc- 

curs a  great  congestion  in  the 
region  of  the  superior  vena 
cava  and  the  tributary  veins 
and  lymphatics.  This  stasis, 
during  a  paroxysm,  causes  not 
only  the  cyanotic  discoloration 
of  the  face  and  mucous  mem- 
branes already  described,  but 
also  a  permanent  distention 
of  the  lymphatic  ducts  and 
blood  vessels,  which  may  be 
seen  in  the  pauses.  Accord- 
ingly, the  faces  of  children 
become  bloated,  particularly 
where  there  is  loose  subcuta- 
neous cellular  tissue;  the  upper 
and  the  lower  eyelids  stand  out  like  pillows,  and  sometimes  the  eyeballs 
protrude  slightly.  In  thin  cliildren  the  contrast  between  the  scant  fat 
of  the  body  and  the  full,  puffy  face  is  often  surprising.  Ha?morrhages 
occurring  during  an  attack,  especially  in  the  conjunctiva,  are  a  conse- 
quence of  the  greatly  increased  pressure  in  the  venous  circulation.  They 
may  be  so  profuse  that  the  sclera,  so  far  as  visible,  appears  blackish  red. 


Facies  pertussea.    Three-year-old  girl. 
Tiilrd  week  of  whooping-cough. 


WHOOPING-COUGH  467 

Rarely,  and  only  in  cachectic  children,  does  the  skin  of  the  face  and  body 
become  blackened  through  extravasations  of  blood.  In  children  with 
visible  veins  on  the  head  and  forehead,  these  vascular  trunks  become 
swollen  as  thick  as  a  raven's  quill. 

Characteristic  of  whooping-cough  is  a  sublingual  vdcer  or  ulcer  of 
the  frtenum.  In  children  that  have  their  lower  incisors,  we  find  a  rhom- 
boid or  lancet-shaped  ulcer  of  the  frenum,  \\'ith  a  thick  white  coating. 
This  ulcer  is  formed  during  paroxysms  by  the  protrusion  of  tongue, 
wliich  at  each  cougliing  effort  swee^DS  its  under  surface  over  the  edges 
of  the  lower  incisors.  The  subhngual  ulcer  occurs  only  in  whooping- 
cough  and  at  times  becomes  an  important  diagnostic  aid.  [This  ulcer  is 
seen  also  in  other  .severe  paroxysmal  coughs  in  young  cliildren  and  is 
not  diagnostic. — La  F.] 

A  physical  examination  after  a  prolonged  duration  of  the  convul- 
sive stage,  reveals  on  percussion  the  signs  of  a  certain  pulmonary  em- 
physema, the  diaphragm  standing  deep,  the  intercostal  spaces  being 
fuller,  and  the  upper  cla\'icular  fossse  vaulted  forward.  On  ausculta- 
tion, especially  immediately  before  a  paroxysm,  we  discover  here  and 
there  a  rhoncus,  a  fine  rattling  rale  wliich  disappears  during  the  attack. 
As  to  the  heart,  pro\Tded  an  existing  pulmonary  emphysema  does  not 
affect  the  conditions  on  percussion,  we  notice  an  increase  of  the  area  of 
cardiac  dulness  towards  the  riglit,  due  to  a  dilatation  of  the  right  ven- 
tricle, frequently  also  an  increased  intensity  of  the  second  pulmonary 
sound,  wliich  is  an  expression  of  increased  pressure  in  the  lesser  circu- 
lation. This  congestion  gives  rise  to  intense  venous  hypersemia  in  the 
whole  respiratory  tract  and  predisposes  to  haemorrhages  that  are  fre- 
quently observed.  0^\-ing  to  small  vascular  ruptures  the  expectorated 
mucus  often  appears  flecked  with  blood. 

According  to  Blumenthal-Hippius  the  condition  of  the  urine  is  said 
to  be  diagnostically  important  even  in  the  catarrhal  stage.  It  is  of  a 
pale  yellow  color^  strongly  acid,  of  a  liigh  specific  gravity  (1.022  to  1.032) 
precipitating  a  large  number  of  crystals  of  free  uric  acid.  The  daily 
quantity  of  uric  acid  secreted  exceeds  the  normal  by  two  or  three  fold. 
Co-existing  febrile  comphcations  very  much  lessen  the  value  of  urinary 
findings  as  specific  symptoms. 

Gh'cosuria  in  whooping-cough  has  been  frequently  reported,  Init 
the  excretion  of  sugar  is  far  from  being  constant. 

With  regard  to  the  condition  of  the  blood  in  whooping-cough  it 
may  be  .said,  according  to  recent  examinations  (F.  Cinia),  that  in  simple 
pertussis  leucocytosis  occurs  at  the  very  inception,  three  times  as  many 
white  blood  corpuscles  as  in  the  normal  condition  being  usually  found. 
The  intensity  of  leucocj'tosis  runs  parallel  to  the  intensity  of  the  dis- 
ease. The  increase  in  the  number  of  leucocytes  is  greater  in  smaller 
children  and  during  comphcations.     With  regard  to  the  increase  of  the 


468  THE    DISEASES   OF   CHILDREN 

several  forms  of  k-ucoeytes  in  the  .sciLse  that  the  increase  of  the  lympho- 
cytes precedes  that  of  the  polymorphonuclear  leucocytes,  or  that  the 
number  of  the  former  decreases  in  proportion  as  the  latter  increase, 
no  definite  law  has  been  observed.  Consequently,  nothing  positive 
can  be  enunciated  concerning  the  pathogenetic  mechanism  of  the 
leucocytosis.  In  doubtful  cases  the  blood  findings  may  be  of  diagnos- 
tic value. 

Examinations  of  the  blood  (by  Crisafi)  in  whooping-cough  are  said 
to  give  positive  iodine  reaction  in  80  per  cent,  of  the  cases.  The  iodo- 
phile  granules  are  present  in  variable  numbers,  especially  in  the  polynu- 
clear  leucocytes,  less  frequently  in  the  eosinophiles  and  rarely  in  the 
lymphocytes. 

Under  certain  circumstances  we  meet  with  variations  from  the 
usual  course  of  pertussis.  Here  belongs  the  frequently  shortened  and 
scarcely  characterized  catarrhal  stage  in  young  infants.  In  such  cases 
the  spasmodic  paroxysms  set  in  rapidly,  often  in  the  first  days  of  the 
illness.  In  cliildren  of  the  first  year  of  life  the  characteristic  stridulous 
inspiration  following  the  succession  of  staccato-hke  coughing  efforts  is 
lacking,  and  the  rarity  or  total  absence  of  deep  inspiration  easily  leads 
to  apnoea  and  to  syncopal  accidents. 

At  the  beginning  of  the  convulsive  stage,  rarely  during  its  whole 
course,  the  paroxysms  at  times  set  in  with  spasmodic  sneezing,  which 
presently  passes  into  a  convulsive  cough,  or,  seldom,  by  itself  constitutes 
the  whole  paroxysm,  and  terminates  with  choking  and  vomiting.  Such 
a  vicarious  sneezing  may  persist  during  the  whole  of  the  whooping- 
cough. 

A  dyspeptic  form,  too,  in  which  gastric  and  intestinal  disturbances, 
along  with  spasmodic  cough,  are  the  saHent  features,  has  been  observed 
by  several  (Jacobson),  but  such  forms  are  dubious. 

Sometimes  the  course  of  pertussis  is  characterized  by  such  mild 
symptoms  that  only  the  existence  of  an  epidemic  and  the  liistory  of  ex- 
posure to  the  infection  enable  us  to  form  a  diagnosis.  The  paroxysms 
are  but  slightly  marked,  the  cough  resembles  the  vigorous  and  repeated 
expiratory  efforts  met  with  in  bronchitis  with  scant  .secretion,  and  the 
whooping  inspiration  is  lacking.  In  adults  such  forms  (coqueluchette, 
forme  fruste)  are  more  frequent  than  in  children.  A  correct  recognition 
of  such  a  case  is  not  merely  diagnostically  important,  but  also  for  the 
reason  that  it  might  easily  become  the  starting-point  of  a  house  epidemic 
and  a  further  spreading  of  the  disease. 

In  its  symptoms  pertussis  at  times  becomes  indefinite,  when  accom- 
panied by  febrile  complications  together  with  great  prostration  or 
considerable  respiratory  disturbances,  and  above  all  by  broncho- 
pneumonic  infiltrations.  In  such  conditions  the  cougliing  spells  lose 
their  characteristic  peculiarities,  and  assume  the  form  of  short,  dyspnoeic 


WHOOPING-COUGH  469 

coughing  efforts.  Now  and  then  the  underlying  affection  betrays  itself 
by  severe  cyanosis  and  jerky  respiratory  efforts  ending  in  syncopal 
attacks. 

The  average  duration  of  wliooping-cough  in  light  uncomplicated 
cases,  varies  between  eight  and  twelve  weeks.  Still  the  majority  of 
cases  exceed  tliis  duration  considerably.  Exacerbations  and  various 
factors  prolong  each  of  the  several  stages.  The  season  of  the  year  at 
which  the  illness  sets  in,  constitution,  etc.,  may  cau.se  the  whooping- 
cough  to  persist  for  months.  Generally,  during  the  warm,  dry  months 
conditions  are  more  favorable.  The  observation  has  been  made  time 
and  again,  that  weeks  and  months  after  the  complete  disappearance  of 
the  characteristic  cough,  a  sUght  cold  or  a  hght  broncliitis  may  revive 
in  the  child  the  typical  cough  of  pertussis.  This,  justly  as  it  seems,  was 
explained  by  the  assumption  of  a  certain  preparation  of  the  nerve- 
paths  involved  in  the  cough  reflex.  The  superior  laryngeal  nerve  is 
regarded  as  one  side  of  the  reflex  arc.  An  abortive,  greatly  shortened 
course  of  pertussis  is  said  to  occur  at  times,  but  it  is  very  rare. 

The  complications  of  whooping-cough  may  be  accounted  for  mainly 
by  the  mechanism  of  the  paroxysms  and  the  lesions  that  may  be  caused 
by  it  directly.  These  include  subconjunctival  haemorrhages,  epistaxis. 
vascular  enlargement  of  the  thyroid  gland,  ulcer  of  the  frenum,  and 
pulmonary  emphysema  due  to  increased  expiratory  pressure.  Some- 
times increased  abdominal  pressure  leads  to  prolapse  of  the  rectum 
and,  in  case  the  inguinal  canal  remains  open,  to  inguinal  hernia. 
Umbilical  hernias,  too,  usually  in  small  children,  may  occur  during  the 
process  of  whooping-cough.  Rupture  of  the  recti  abdominalis  muscle  is 
a  complication  of  rare  occurrence. 

Hemorrhages  of  the  mucous  membranes  of  the  nose,  mouth  and 
bronchi  and  multiple  haemorrhages  of  the  skin  may  he  regarded  as 
effects,  partly  of  the  greatly  increased  intravenous  pressure  and  partly  of 
causes  affecting  and  alternating  the  vascular  walls.  But  it  is  question- 
able whether  increased  pressure  alone  is  able  to  produce  haemorrhages. 
Haemorrhage  also  may  occur  from  suppurating  foci,  from  the  subUngual 
ulcer,  from  moist  eczema,  from  catarrhal  areas  in  the  nose,  and  from 
the  ruptured  drum  membrane  when  the  middle  ear  is  involved.  In 
subconjunctival  haemorrhages  a  preceding  lesion  of  the  vascular  wall 
(conjunctivatis)  can  be  assumed  as  a  causative  factor.  In  young  cliildren 
bleeding  from  the  mouth  is  often  due  to  biting  of  the  tongue  during  a 
paroxysm. 

On  account  of  their  frequency  and  importance  in  the  prognosis  of 
whooping-cough,  complications  of  the  respiratory  tract  deserve  the 
greatest  interest.  They  occur  mostly  in  the  convulsive  stage.  If  in  the 
intervals  between  the  attacks  the  general  condition  of  the  patient  is  not 
good,  if  between  the  paroxysms  there  is  dyspnoea,  irritative  cough,  and 


470  THE   DISEASES   OF   CHILDREN 

rales  audible  at  a  distance,  we  may  infer  that  there  is  some  complica- 
tion of  the  respiratory  organs.  If  there  is  also  fever,  then  there  is  a 
capillary  broncliitis  or  a  pulmonary  consohdation,  as  will  be  revealed  by 
physical  examination,  wliich  will  show  characteristic  signs.  As  the 
Ughtest  complication  we  find  a  diffuse  catarrh.  From  tliis  may  develop, 
slowly  or  rapidly,  a  catarrh  of  the  finer  bronchi  or  a  lobular  infiltration 
of  the  lung. 

Infancy  especially  predisposes  to  such  comphcating  pulmonary 
affections,  so  that  infants  a  few  months  old  rarely  escape.  Cliildren  of 
any  age  affected  with  whooping-cough,  who  are  weak  with  lessened 
resistance,  or  who  are  weakened  by  preceding  or  present  constitutional 
diseases,  have  their  chances  of  recovery  vastly  diminished  by  broncho- 
pneumonia. Still  the  prognosis  is  never  totally  hopeless,  not  even  for 
the  youngest  infants.  The  duration  of  bronchopneumonia  is  prolonged 
by  the  severity  of  the  underlying  disease  and  often  by  more  or  less 
frequent  relapses. 

During  the  course  of  whooping-cough,  we  meet  quite  frequently 
Anth  symptoms  that  can  be  referred  to  titberculosis  of  the  pulmonary 
and  bronchial  lymph-nodes.  These  develop  either  primarily,  betraying 
themselves  by  a  certain  debiUty  of  the  organism;  by  emaciation;  by  a 
faded  gray  color  of  the  skin;  by  febrile  manifestations,  and  objectively 
by  impaired  pulmonary  resonance  in  one  or  both  intrascapular  spaces 
(tuberculous  infiltration  of  the  bronchial  lymph-nodes);  or  they  pass  on 
to  a  protracted  and  relapsing  lobular  pneumonia  and  thus  become  an  early 
manifestation  of  latent  tuberculosis.  Tuberculous  compUcations  of  the 
lungs  are  uncommonly  frequent.  These  are  almost  never  wanting  in 
the  post-mortem  examinations  of  whooping-cough  patients.  Still  they 
may  reach  a  state  of  symptomatic  quiescence  and  later  on,  after  days 
and  years,  may  terminate  with  an  abrupt  manifestation  of  mihary  tuber- 
culosis or  tuberculous  meningitis. 

Chronic  tuberculosis  of  the  pulmonary  or  bronchial  lymph-nodes 
is  often  encountered  as  a  sequel  when  all  the  symptoms  of  pertussis 
have  disappeared.  A  goodly  number  of  tuberculous  children  date  their 
affection  back  to  whooping-cough. 

Another  typical  comphcation  of  pertussis  is  bronchiectasis,  a  cylin- 
drical dilatation  of  the  bronchi,  mostly  multiple  and  accompanied  by 
scar  formation  in  the  proximity.  About  the  termination  of  bronchiec- 
tasis in  cluldhood,  not  much  is  known.  The  significance  of  its  progress 
is  that  it  is  rarely  unaccompanied  by  the  disea.ses  of  the  lungs.  Influ- 
enza may  set  in  during  the  course  of  whooping-cough;  bacteriological 
examinations  by  Jehle  showing  the  presence  of  the  influenza  bacillus  in 
twenty-four  cases  of  pertussis  always  in  the  lungs  and  twelve  times  in 
blood.  Mediastinal  or  subpleural  emphysema,  apt  to  spread  in  the 
subcutaneous  tissue   over   the   upper   half  of   the  body,   is   frequently 


WHOOPIXG-COUGH  471 

observed,  disappearing  after  a  shorter  or  longer  time.  In  such  cases  the 
skin  produces  a  sensation  somewliat  hke  an  air  cushion  and  crackles  on 
palpation.  Pleuritis  of  a  serous  or  purulent  nature  occurs  very  rarely 
as  a  complication  of  pertussis. 

Among  the  comphcations  involving  the  circulatory  organs  the  mild- 
est ones  are  the  previously  mentioned  congestive  phenomena.  As  a 
result  of  congestion  in  the  lesser  circulation  there  may  occur  a  primary 
dilatation  with  subsequent  hypertrophy  of  the  right  ventricle.  Only  in 
greatly  reduced  children  where  the  \'iolent  paroxysms  of  coughing  are 
excessive  is  this  hypertrophy  wanting  and,  the  heart  not  being  equal 
to  the  demands  in  such  cases,  sudden  heart  failure  may  occur.  Other 
complicating  heart  diseases,  such  as  endocarditis  and  pericarditis,  are 
very  rare. 

On  the  other  hand,  the  peripheral  vessels  may  at  times  sustain  an 
injury  from  the  toxin  of  whooping-cough.  While  no  histological  proof 
for  such  alteration  of  the  vascular  wall  has  as  yet  been  forthcoming,  cer- 
tain chnical  observations  make  it  appear  almost  positive.  Thus  in  the 
course  of  pertussis  in  weak  cliildren  we  notice  a  distinct  cedema  of  the 
subcutaneous  cellular  tissue,  although  neither  the  heart  nor  the  urine 
furnishes  us  anj'  data  for  a  satisfactory  explanation.  Moreover,  we  are 
inclined  to  assume  that  the  factor  of  congestion  alone  is  hardly  sufh- 
cient  for  the  production  of  cutaneous,  subconjunctival,  and  intracranial 
ha?morrhages,  but  that  the  vascular  walls  must  have  a  weakened  power 
of  resistance  to  be  ruptured  by  increased  blood  pressure. 

Among  the  nervous  complications  and  sequelte  of  whooping-cough, 
convulsions  are  the  most  frequent.  Their  typical  course  is  character- 
ized by  the  occurrence  of  clonic-tonic  spasms  during  the  convulsive  stage 
and  following  a  coughing  paroxysm  involving  either  a  Umited  muscular 
area,  or,  as  in  epileptic  attacks,  all  of  the  voluntary  muscles,  especially 
those  of  the  extremities.  Such  eclamptic  seizures  usually  follow  the 
paroxysms,  but  at  times  they  may  occur  between  and  independently  of 
the  cougliing  attacks.  The  convulsions  are  always  multiple,  a  single 
attack  hardly  ever  occurring.  Frequently  there  coexists  a  fever. 
Between  the  several  spasmodic  seizures  wliich  are  always  attended 
with  loss  of  consciousness  there  may  persist  psycliical  disturbances, 
stupor,  and  rigidity  of  the  neck.  The  convulsions  occur  usually  in  chil- 
dren during  the  first  year  of  hfe,  who  in  other  ways  show  a  great  irri- 
tability of  the  cortical  centre.  They  indicate  a  severe  and  dangerous 
compUcation.  During  and  between  the  eclamptic  seizures,  a  remission 
of  the  whooping-cough  attacks,  both  in  number  and  intensity,  is 
observed. 

French  medical  writers  mention,  besides  the  convulsions  (convul- 
sions externes),  also  spasm  of  the  glottis  (convulsions  internes)  as  a 
frequent  comphcation  of  whooping-cough. 


472  THE   DISEASES   OF   CHILDREN 

Clinically  a  typical  picture  of  meningitis  complicating  pertussis  is  sel- 
dom met  with.  The  rare  occurrence  of  tuberculous  meningitis  is  in  strik- 
ing disproportion  to  the  frequency  of  tuberculous  affections  of  the  lungs. 

Cerebral  paralyses,  either  hemiplegic  or  diplegic,  have  recently  be- 
come known  as  more  frequent  complications  of  whooping-cough  (Hock- 
enjos,  Valentin,  Neurath).  They  appear  under  the  usual  types  of  cere- 
bral paralysis,  either  as  monoplegias,  hemiplegias,  diplegias,  sometimes 
combined  with  bulbar  symptoms,  or  paralysis  of  the  muscles  of  the  eye 
or  of  the  sense  organs. 

In  the  category  of  cerebral  paralyses  probably  belong  also  disease 
pictures  suggestive  of  nuiltiple  sclerosis  following  pertussis.  Fried- 
reich's ataxia  likewise  has  been  observed  after  whooping-cough.  Among 
the  psycliic  disturbances  manifesting  themselves  during  the  course  of 
pertussis  we  find  insanity  (in  the  form  of  hallucinations),  complete 
imbecility  (as  shown  by  Baginsky's  illustrative  examples),  symptoms  of 
pavor  nocturnus,  etc.  W.  Konig  saw  permanent  idiocy  follow  whoop- 
ing-cough, but  usually  the  psycliic  complications  of  pertussis  are  of  a 
transitory  character. 

Sudden  bhndness  after  whooping-cough  as  a  result  of  haemorrhages 
into  the  anterior  chamber  or  retinal  detachment  or  central  lesion, — the 
\'isual  power  usually  soon  returning, — auditory  disturbances  due  to  a 
compUcating  otitis  media  or  to  a  direct  lesion  of  the  nervous  apparatus, 
and  disturbances  of  sensibility  are  among  the  sense  disturbances  that  at 
times  comphcate  whooping-cough. 

Among  the  spinal  cord  disturbances  may  be  mentioned  a  flaccid 
paralysis  of  distinct  spinal  character,  which  however  cannot  be  readily 
explained  (htemorrhage,  myeUtis,  poliomyelitis).  Pains  in  the  legs  and 
loins,  disturbances  of  sensation,  difficulty  in  the  evacuation  of  the  bowels 
or  urine  may  complicate  the  paralyses  (Bernhardt,  Luisada).  Also  poly- 
neuritis (Faidherbe,  Aldrich),  Landry's  ascending  paralysis  (Mobius, 
Hagedorn),  and  a  case  of  so-called  pseudotabes  (Simionesco)  have  been 
observed  after  whooping-cough. 

The  anatomy  of  the  cerebral  complications  of  pertussis  has  recently 
been  made  the  subject  of  close  investigation  (Neurath).  Certain  nega- 
tive necropsy  findings  in  well-marked  chnical  pictures,  suggestive  now 
of  hsemorrhagic  effusions  or  embohsm,  now  of  encephalitis,  and  again  of 
meningitis,  have  given  rise  to  the  supposition  that  liistological  changes 
might  be  found  wMch  escape  the  free  eye.  It  is  undeniable  that  intra- 
cranial haemorrhages  are  clinically  more  frequently  diagnosed  than 
anatomically  verified.  In  such  hsemorrhages  a  lesion  of  the  vascular 
wall  (called  aneurysmatic  dilatations  by  Vidal)  plays  a  greater  role  in 
pathogenesis  than  the  increased  intravenous  pressure. 
*  Neurath  found  in  a  series  of  cases  which  during  Ufe  presented 
symptoms  mostly  of  cerebral  irritation,  a  pronounced  meningeal  infil- 


WHOOPING-COUGH  473 

tration  (mononuclear  leucocj-tes),  hypersemia,  and  meningeal  ha'mor- 
rhage  from  inflammation — fintlings  analogous  to  those  obtained  in  other 
acute  infectious  diseases  such  as  typhoid  fever,  scarlet  fever,  and  sepsis. 
He  is  incUned  to  attribute  to  this  meningitis  simplex  a  pathogenetic 
explanation  of  the  development  of  a  number  of  cerebral  complications, 
in  addition  to  the  other  anatomical  data  (emboUsm,  haemorrhage, 
encephaUtis,  etc.).  This  assumption  seems  to  find  a  support  in  the  re- 
sults obtained  by  Bertolotti  and  others  in  lumbar  punctures  systemati- 
cally performed  on  cliildren  suffering  with  whooping-cough.  They  found 
the  puncture  fluid  to  abound  in  mononuclear  leucocytes. 

With  regard  to  the  digestive  tract,  apart  from  a  prolapse  of  the 
rectum  mentioned  pre%'iously,  we  find  now  and  then  gastric  and  intes- 
tinal catarrhs.  Acute  nephritis,  sometimes  ushered  in  \\-ith  fever,  is  rel- 
atively seldom  met  with,  but  it  may  occur  and  be  attended  with  uraemic 
symptoms.  A  compUcating  otitis  media,  occurring  during  the  cour.se 
of  pertussis,  probably  starts  from  the  nasopharyngeal  space.  Among  the 
cutaneous  affections  we  find,  besides  the  hemorrhages  already  mentioned, 
sometimes  erythema,  pemphigus,  urticaria-like  efflorescences  (even  non- 
medicamentous).    They  may  exhibit  a  hsemorrhagic  character. 

Constitutional  disturbances,  antemia  and  scrofula,  are  not  rarely 
the  sequelae  of  whooping-cough.  Their  severity  is  in  proportion  to  the 
severity  and  duration  of  the  basal  disease  and  the  intercurrent  affections. 

Whooping-cough  is  often  found  associated  -nith  other  acute  infec- 
tious diseases.  TMs  fact  can  be  accounted  for  only  by  an  increased 
disposition,  due  either  to  the  opening  up  of  portals  of  infection  or  to  a 
general  weakening  of  the  resisting  powers.  Especially  in  hospitals  mth 
insufficient  accommodation  for  isolation,  certain  disease  combinations 
are  apt  to  occur.  Foremost  among  the  latter  are  measles,  which  more 
frequently  follows  than  precedes  pertussis.  Varicella  is  likewise  often 
a  compUcation,  whereas  scarlet  fever  is  rarely  found  associated  with 
pertussis. 

The  diagnosis  of  whooping-cough  in  typical  cases  is  easy,  its  symp- 
tomsaud  course  as  a  rule  being  so  characteristic  that  the  history  alone 
suffices.  Still  in  many  cases  conchtions  have  to  be  considered  wliich 
may  simulate  whooping-cough. 

The  most  important  symptom  of  whooping-cough  is  the  typical 
paroxysm.  A  spasmocUc  cough  caused  by  tuberculosis  of  the  broncliial 
glands  may  in  numerous  cases  suggest  a  staccato  cough.  In  such  cases 
the  course. of  the  disease  is  important  for  a  differential  diagnosis.  In 
whooping-cough  the  cough  increases  in  a  typical  manner  from  the  onset 
through  the  catarrhal  stage  (1  to  2  weeks)  up  to  the  beginning  of  the 
paroxysms,  the  latter  usually  terminating  with  vomiting.  During  the 
intervals  the  child  feels  well.  The  lungs,  on  examination,  do  not  yield 
any  results  differing  from  the  normal.     On  the  other  hand,  in  enlarge- 


474  THE    DISEASES   OF   CHILDREN 

ment  of  the  broncliial  glands  there  is  no  chniax,  the  cougliing  attacks 
preventing  the  recognition  of  the  distinct  whooping  ins])irations.  Vom- 
iting is  rare,  and  the  cliildren  convey  the  impression  of  being  constitu- 
tionally stricken  individuals  (scrofulosis),  and  frequently  exhibit  hectic 
fever,  while  an  examination  of  the  thorax  reveals  dulness  and  bronchial 
breathing  between  the  shoulder  blades. 

A  pertussis-hke  hysterical  imitation  cough  may  be  distinguished 
from  true  whooping-cough  by  the  hysterical  stigmata  of  the  afflicted 
children,  by  the  cessation  of  cougli  during  sleep,  by  the  absence  of 
certain  signs  of  pertussis  (pufied  face,  subhngual  ulcer).  Whooping- 
cough  may,  without  much  difficulty,  be  distinguished  from  a  reflex 
cough  caused  by  hypertrophy  of  the  tonsils,  and  from  the  symptoms 
provoked  by  inhalation  of  foreign  bodies. 

Finally,  the  lustory  will  exclude  the  existence  of  pertussis  in  those 
cases  in  which  a  catarrh,  coming  on  long  after  whooping-cough  has  been 
cured,  causes  paroxysm-hke  attacks  by  bringing  into  activity  the  ner- 
vous paths  wliich,  through  the  ordeal  of  pertussis,  have  undergone  a 
certain  training. 

The  diagnosis  of  whooping-cough  can  be  safely  established  by  the 
physician  when  he  has  observed  a  typical  paroxysm.  We  have  certain 
methods  of  producing  an  attack.  For  instance,  by  introducing  a  spa- 
tula into  the  pharynx  we  may  provoke  choking  and  vomiting.  But  con- 
tinued pressure  with  the  finger  on  the  trachea  or  thyroid  cartilage  usually 
sufhces.  Tickhng  the  nasal  mucous  membrane  or  of  the  external  audi- 
tory meatus  accomplishes  the  same  result. 

Enumerating  the  positive  data  to  be  taken  into  consideration  for  a 
dia-gnosis  of  whooping-cough,  we  find: 

1.  After  a  shghtly  characteristic  catarrhal  stage  the  typical  parox- 
ysms set  in  with  spasmodic  expirations  and  few  intervening  crowing 
inspirations,  terminating  with  the  production  of  a  tenacious,  glairy 
mucus.  Cliildren  in  the  first  years  of  life  commonly  swallow  their  spu- 
tum.   In  whooping-cough  the  gagging  and  vomiting  voids  the  sputum. 

2.  The  cough  is  often  more  frequent  by  night.  It  may  be  caused 
by  hearing  and  seeing  a  paroxysm  in  another  cliild. 

3.  Results  of  an  objective  chnical  examination  are:  lung  signs  neg- 
ative in  spite  of  the  violent  coughing;  permanent  lymphatic  congestion 
(puffed  face,  swelUng  of  the  eyehds),  subhngual  ulcer,  subconjunctival 
haemorrhages. 

4.  Urinary  symptoms  (Blumenthal-Hippius),  blood  findings  (leuco- 
cytosis). 

5.  Exposure  to  infection,  existence  of  an  epidemic,  same  disease  in 
brothers  or  sisters. 

6.  Failure  of  antispasmodic  medication  (belladonna,  morphine)  in 
the  customary  doses,  so  long  as  the  disease  is  on  the  ascent. 


WHOOPING-COUGH  475 

Diagnostic  difficulties  arc  encountered  at  the  beginning  of  the  ca- 
tarrhal stage,  as  anything  typical  of  whooping-cough  is  absent.  In  this 
phase  an  examination  of  the  urine  and  of  the  blood  and  perhaps  the 
liistory  of  exposure  to  infection  may  facilitate  the  diagnosis. 

In  very  young  children  in  whom  the  paroxysms  pursue  an  atypical 
course,  without  crowing  inspiration,  and  in  the  so-called  "  formes  frustes  " 
of  pertussis,  and  finally  when  severe  complications  frustrate  the  parox- 
ysms (pneumonia,  convulsions),  the  history  may  at  times  enable  us  to 
make  a  diagnosis,  yet  it  is  frequently  impossible  to  be  positive  at  once. 

Prognosis. — For  vigorous  older  cliildren  whooping-cough  is,  on 
the  whole,  a  harmless  disease;  but  for  the  weak,  chronically  sick,  and 
especially  very  young  cliildren,  it  is  often  of  fatal  significance.  Becau.se 
the  younger  ages  are  largely  involved,  the  mortality  from  whooping- 
cough  is  statistically  rather  high.  In  the  years  1895  to  1901,  according 
to  the  statistical  year-book  of  Vienna,  15,711  cases  were  reported  as 
having  occurred  in  that  city,  of  wluch  1052,  or  6.6  per  cent.,  terminated 
fatally. 

A  comparison  of  whooping-cough  with  measles  and  scarlet  fever 
for  the  three  years  1899  to  1901  furnishes  the  following  results: 

Whooping-cough 6469  cases 444,  or  6 .  86  per  cent.  died. 

Measles 37,257  eases 1778,  or  4.77  per  cent.  died. 

Scarlet  fever 7176  cases 631,  or  8.79  per  cent.  died. 

According  to  Presl  the  aggregate  mortahty  from  the  following  four 
infectious  diseases  for  Austria  during  the  year  1883  was: 

Measles 0 .  45  per  cent. 

Scarlet  fever .- 0 .  61  per  cent. 

Whooping-cough 1 .  09  per  cent. 

Diphtheria 1 .41  per  cent. 

According  to  the  above  data  whooping-cough  occupies  the  second 
place  among  the  more  dangerous  infectious  diseases.  The  late  sequelfe 
of  pertussis  (tuberculosis)  evidently  being  left  out  of  consideration,  the 
■  mortahty  from  whooping-cough  probably  is  much  liigher. 

As  an  illustration  of  the  greater  danger  of  exposure  to  infection  dur- 
ing the  earlier  ages  we  present  the  following  Menna  statistics  for  the 
years  1899  to  1901: 

First  year  of  life 1242  cases 322  or  25.3  per  cent.   died. 

Second  to  fifth  year 3139  ca.ses 214  or    6.8  per  cent.   died. 

Sixth  to  tentli  year 1926  cases 75  or    3.9  per  cent.    died. 

Eleventh  to  fifteenth  year 135  case.s 10  or    7.4  per  cent.    died. 

Sixteenth  to  twentieth  year 12  cases 0 0  .    died. 

Twenty-first  to  twenty-fifth 2  cases 0 0 died. 

Twenty-sixth  year 13  cases 0 0 died. 

Infants  are  generally  the  most  endangered.  Owing  to  frequent 
vomiting,  their  nutrition  is  easily  affected.     Moreover,  they  are  exposed 


476  THE   DISEASES   OF   CHILDREN 

to  pulmonary  complications,  resulting  in  a  mortality  of  95  per  cent. 
However,  the  very  youngest  of  infants  (in  tlie  first  weeks  of  life)  seem  to 
endure  the  disease  somewhat  better — a  fact  observed  by  Porak  and 
Durante  during  a  domestic  epidemic  which  had  broken  out  in  a  pavihon 
intended  for  premature  infants. 

Of  the  greatest  prognostic  importance  is  the  intensity  of  the  affec- 
tion, wlaich  may  be  influenced  by  various  factors,  such  as  reaction  of 
the  individual,  character  of  the  epidemic,  season  of  the  year,  and  hy- 
gienic conditions.  An  indication  of  the  intensity  of  the  disease  is  the 
number  of  attacks.  According  to  Trousseau,  over  sixty  attacks  a  day 
constitute  a  bad  prognosis.  Periods  entirely  free  from  attacks  along 
with  undisturbed  well-being  are  the  signs  of  a  favorable  normal  course 
of  the  disease. 

From  the  above-mentioned  complications,  besides  pulmonary  affec- 
tions, a  fatal  termination  may  be  induced  by  cardiac  insufficiency, 
great  losses  of  blood  (epistaxis),  and  cerebral  complications  (haemor- 
rhage). 

Every  intercurrent  infectious  disease  (measles,  varicella,  diphtheria) 
diminishes  the  prospects  of  a  cure,  both  for  the  whooping-cough  and 
the  complications,  even  if  a  momentary  diminution  of  the  daily  number 
of   paroxysms   simulates   an   ameUoration. 

The  prophylaxis  of  whooping-cough  in  its  main  features  is  based 
on  the  details  of  its  course  and  of  the  infection.  The  most  important 
precautionary  measures  include  the  early  segregation  of  the  afflicted 
child  from  the  healthy.  As  the  symptoms  of  the  initial  stage  are  usually 
ambiguous  and  not  characteristic  and  as  they  must  certainly  be  regarde'd 
as  infectious,  the  pressure  of  an  epidemic  of  pertussis  should  warn  us  to 
look  upon  every  cougliing  child  as  a  whooping-cough  suspect. 

The  prophylactic  measures  belong  partly  to  the  domain  of  pubUc 
hygiene,  and  partly  to  the  individual  or  family.  Above  all,  every  cliild 
suspected  of  whooping-cough  should  be  kept  away  from  the  school, 
nursery,  or  kindergarten,  and  its  brothers  and  sisters,  as  well  as  the  other 
inmates  of  the  house,  should  be  segregated  during  the  entire  duration 
of  the  disease,  unless  they  have  had  whooping-cough  themselves  and 
are  removed  from  all  contact  with  the  patient.  Moreover,  superinten- 
dents of  pubhc  playgrounds  and  parks  should  exclude  cliildren  sick 
with  whooping-cough,  or  reserve  grounds  for  them  provided  with  cus- 
pidors containing  disinfectants.  Separate  compartments  in  railroad 
cars,  as  recommended  by  many,  would  be  a  desirable  arrangement, 
whereas  the  u.se  of  pubhc  vehicles  should  be  proliibited. 

Private  prophylaxis  includes  strict  isolation  of  the  affected  child 
from  the  healthy  ones  in  order  to  prevent  the  spread  of  the  ^disease.  It 
might  not  be  unwdse  in  certain  cases,  provided  the  age  is  sufficient  and 
constitution  good,  to  allow  the  brothers  and  sisters  to  be  visited  by  the 


WHOOPING-COUGH  477 

infection,  in  order  to  guard  against  the  possibility  of  a  future  invasion 
of  the  house  after  months  or  years.  Indirect  transmission,  through 
soiled  objects,  must  likewise  receive  proper  attention. 

Prophylaxis  also  includes  protection  from  a  whooping-cough  epi- 
demic in  case  of  change  of  location  of  the  stricken  indi^^duals.  Thus 
during  the  hot  season  many  summer  resorts  and  sanatoria  that  are 
largely  frequented  are  apt  to  be  endangered,  so  that  the  exclusion  of  a 
newly  arrived  child  stricken  with  whooping-cough  is  a  justifiable  measure 
of  self-protection. 

When  received  at  a  hospital  for  contagious  diseases,  every  child 
suspected  of  whooping-cough  must  be  kept  away  from  the  wards  in- 
tended for  other  diseases,  and  thus  by  adequate  isolation  a  domestic 
epidemic  be  guarded  against,  as  it  is  well  known  how  fatal  any  com- 
bination -ftith  other  infectious  diseases  may  become.  Considering  the 
high  whooping-cough  mortahty  in  hospitals,  the  admission  of  children 
stricken  with  pertussis  should  wisely  be  confined  to  such  cases  as 
come  from  the  very  lowest  of  social  and  hygienic  surroundings  and 
for  such  individuals  hospital  treatment  would  indeed  signify  a  relative 
ameUoration. 

When  the  whooping-cough  has  run  its  course,  all  the  rooms  should 
be  thoroughly  disinfected. 

Treatment. — The  therapy  of  pertussis  rests  nowadays  on  mere 
empiricism.  An  etiological  treatment  was  attempted  by  Manicatide 
(whooping-cough  serum),  but  the  theoretical  basis  of  his  curative  serum 
s  still  very  questionable  and  practical  results  of  a  convincing  nature  are 
wanting.  Drug  treatment  has  at  its  disposal  an  arsenal  of  means  that 
increases  from  one  day  to  another.  Theoretical  considerations,  adver- 
tisements, a  personal  Uking  for  a  certain  remedy,  etc.,  are  frequently 
the  determining  factors  that  bring  the  highsounding  recommendation 
accompanying  the  latest  drug.  For  the  valuation  of  the  efficacy  of  a 
medicament  proper  attention  must  be  paid  to  the  stage  of  the  disease  at 
which  the  remedy  is  tried  and  to  the  external  factors  influencing  the 
disease,  such  as  hygiene,  cUmate,  etc. 

Of  especial  value  for  the  treatment  of  whooping-cough  are  hygienic 
measures.  In  a  fresh,  warm,  dust-free  atmosphere  without  drafts,  a 
numerical  decrease  of  paroxysms  maj'  alwaj's  be  observed.  A  rational 
fresh-air  treatment  without  mo^^ng  about  much  and  where  all  chance 
of  chilhng  is  carefully  avoided  usually  brings  about  an  improvement; 
nay,  often  the  paroxysms  disappear  so  rapidly  that  an  abortive  course 
suggests  itself.  The  much-vaunted  change  of  air  is  of  value  only  if  it  is 
equivalent  to  an  improvement  of  the  chmatic  conditions.  During  the 
inclement  season  or  when  comphcations  and  febrile  temperatures  pre- 
clude out-door  hfe,  the  so-called  two-room  system  is  recommended,  one 
room  being  thoroughly  aired  while  the  sick  child  stays   in   the  other. 


478  THE   DISEASES   OF   CHILDREN 

By  a  proper  ventilation  of  the  bedroom  at  night  any  increase  in  the  num- 
ber of  paroxysmal  attacks  during  the  night  can  always  be  avoided.  The 
temperature  of  the  room  must  not  be  allowed  to  fall  below  52°  F.  nor  to 
exceed  60°F.  The  child  is  to  be  kept  in  beti  if  the  course  of  the  disease  is 
very  severe  and  debilitating,  and  particularly  if  there  are  febrile  compli- 
cations. During  the  cool  season  it  is  often  advisable  to  warm  the  night 
clothes  and  the  bed  during  the  night. 

The  clothing  of  tlue  cliild  afflicted  with  whooping-cough  should 
correspond  to  the  season  of  the  year  and  not  depart  therefrom  in  any 
direction.  The  fastening  of  the  clothes  must  be  such  that  suspenders, 
braces,  strings,  bands,  or  straps  do  not  interfere  with  the  respiratory 
function  and  the  mechanism  of  cougliing. 

It  is  important  to  counteract  any  failure  of  the  nutritive  condition 
by  a  judicious  regulation  of  the  meals,  both  with  regard  to  their  frequency 
and  quantity  and  quality.  All  crusted,  hard,  too  strongly  tasting  or 
smelhng  food  (which  gives  rise  to  paroxysms)  must  be  avoided.  Too 
copious  meals  easily  incite  coughing.  Frequent  small  meals  are  there- 
fore advisable.  Many  children  can  readily  retain,  without  fits  of  cough- 
ing, food  of  the  consistency  of  gruel  or  pap,  while  others  do  better  mth 
more  liquid  food.  This  factor  must  not  be  left  out  of  consideration. 
The  best  time  for  administering  food  is  after  a  paroxysm.  Medication 
recommended  for  the  whooping-cough  should  display  either  an  expec- 
torant influence  or  an  antizymotic  dr  antispasmodic  action.  With  re- 
gard to  the  mode  of  application,  it  may  consist  of  inhalations,  insuffla- 
tions, pencilling  of  the  pharynx,  nose,  or  larynx,  embrocations,  internal 
remedies,  and  in  physical  curative  methods. 

For  inhalation  carbohc  acid  is  much  used,  in  a  i  to  2  per  cent,  solu- 
tion, by  means  of  an  inhahng  apparatus  or  by  suspending  cloths  dipped 
into  a  10  per  cent,  solution;  aqua  picea,  oleum  terebinthina^  hgnosul- 
phite,  salicylic  acid  (J  to  2  per  cent.),  thymol  (0.02  per  cent.),  benzol, 
(0.01  per  cent.),  naphthahn,  chloroform  (2  to  4  drops  in  a  cup  of  warm 
water),  formaUn  (hygeia  lamp),  cypress  oil  (according  to  Soltmann  10 
to  15  drops  of  a  20  per  cent,  alcohohc  solution  to  be  dripped  on  bed 
■    clothes  and  underwear),  etc. 

Insufflations  of  boric  acid,  benzoin,  sodium  sozo-iodolate,  orthoform, 
quinine  (1  :  magn.  ust.  10),  are  on  account  of  their  cough-inciting  proj)- 
erties  a  two-edged  medicament:  hkewise  pencilhng  with  solutions  of 
quinine  (10  per  cent.),  resorcin  (2  to  3  per  cent.),  cocaine  (10  to  20  per 
cent.),  and  subUmate  (one  per  cent.)  wlaich  may  be  used  for  older  chil- 
dren. Among  the  external  methods  of  treatment  are  included  inunction 
with  antitussin  (difluor  do  phenyl,  a  piece  of  the  size  of  a  pea  over  the 
skin  of  the  back)  and  the  administration  of  enemata  (quinine). 

More  frequent  and  usually  more  sviccessful  is  the  use  of  the  internal 
remedies,  as  an  auxiliary  of  which  sometimes  one  or  the  other  of  the 


WHOOPING-COUGH  479 

above  methods  is  employed.  The  transitory  success  of  the  medicaments 
recommended  is  often  observed  and  makes  a  repeated  change  of  the 
prescription  advisable,  but  none  of  these  insures  any  safe  and  lasting 
benefit. 

In  the  catarrhal  stage  mild  expectorants  bring  relief — liquor  am- 
nion, anisat.,  senega,  ipecacuanha,  etc.;  when  the  cough  is  more  intense, 
such  antispasmodics  as  are  customary  for  cliildhood  (as  althjea,  bella- 
donna, and  very  small  doses  of  codeine  or  morphine).  In  the  paroxys- 
mal stage  we  may  select  one  or  the  other  of  the  antizymotics  and  nar- 
cotics. 

A  favorite  remedy,  in  use  for  a  long  time,  is  quinine  and  its  deri- 
vates:  quinine  [sulphate  of  quinine  0.05  to  0.07  Gm.  (1  gr.)  for  children 
below  one  year;  0.07  to  0.15  Gm.  (1-2  gr.)  for  the  second  and  tliird 
years;  0.15  to  0.25  Gm.  (2-3J  gr.)  for  the  fourth  year  and  above,  one 
powder  three  times  daily];  euquinine  [tasteless,  0.1  to  0.5  Gm.  (1^-7^  gr.) 
three  times  daily];  aristochin  [three  times  daily  as  many  decigrams  as 
the  cliild  is  years,  maximum  daily  dose  1.2  Gm.  (18  gr.)].  The  quinine 
preparations  are  best  given  in  full  doses  for  three  days,  after  which  in 
half  doses  for  six  days,  and  then  discontinued  for  a  few  days.  They 
may  also  be  administered  in  suppositories,  to  spare  the  stomach. 

Among  remedies  having  an  action  similar  to  that  of  quinine,  but  a 
more  distinct  one  on  the  nervous  system  and  the  heart,  and  favorably 
influencing  the  number  of  paroxysms,  may  be  mentioned:  antipyrin, 
tussol  [amygdalate  of  antipyrin,  0.05  to  0.5  Gm.  (1-72  gr-)  three  times 
daily],  citrophen,  salipyrin,  antispasmin  (1.0  :  aqu.  amygdal.  dulc.  10.0, 
three  to  four  times  daily  5  to  20  drops),  pertussin  (3  to  4  teaspoonfuls  a 
day)  coclussin  (10  to  25  drops  several  times  a  day).  The  antispasmodics 
are  frequently  used  as  whooping-cough  remedies,  among  wliich  bella- 
donna and  its  derivates  are  the  most  prominent. 

Belladonna  often  effects  a  surprising  reduction  in  the  curve  of  at- 
tacks, but  fails  after  prolonged  use.  At  the  first  sign  of  any  symptoms 
of  intoxication  it  is  advisable  to  discontinue  the  preparation  (vertigo, 
tickhng  in  the  throat,  dilatation  of  the  pupils).  Belladonna  may  be 
used  by  itself  [pul.  radic.  belladonna^  0.1  Gm.  (1|  gr.)  or  with  sulphate  of 
quinine,  0.5  Gm.  (7A  gr.)  sacch.  2.0  Gm.  (30  gr.),  di\ided  into  ten  powders, 
one  powder  two  to  three  times  daily;  or  its  infusion,  0.5-1.0  to  180.0 
(7^-15  gr.  to  6  oz.),  a  small  spoonful  every  two  to  three  hours;  extract  of 
belladonna,  0.02-0.1  to  10.0  c.c.  (J-li  gr.  to  2h  dr.)  of  aqu.  amygdid. 
dulc,  10  drops  every  three  hours,  or  extract  belladonnte  0.1  Gm.  (li 
gr.),  sacchari  2.0  Gm.  (h  dr.),  divided  into  ten  powders,  3  to  4  powders 
daily;  also  atropine,  0.005  to  100.0  c.c.  (tV  gr.  to  3J  dr.)  of  water,  twice 
daily  1  teaspoonful.  A  sedative  action  on  the  whooping-cough  is 
exercised  by  the  bromides,  1-3  Gm.  (15-45  gr.)  a  day;  bromofonn,  for 
infants  3  to  4  drops,  for  older  children  5  to  7  drops,  three  to  four  times 


480  THE   DISEASES   OF   CHILDREN 

daily;  fluoroforni,  a  2  to  2^  per  cent,  aqueous  solution;  a  coffee — small 
— or  teaspoonful  according  to  the  age  three  to  four  times  a  day.  Thymo- 
bromal  (a  maceration  of  thyme  herb,  folia  castanese  vescae,  radices 
Senegal,  to  wliich  bromoform  is  added,  15  to  20  drops  daily). 

If  the  cause  of  the  disease  is  severe  and  all  the  attempted  remedies 
prove  inefficient,  we  have  to  resort  to  morphine  and  the  allied  alkalies, 
especially  when  it  is  necessary  to  procure  rest  for  the  cliild  after  several 
sleepless  nights  with  many  paroxysms.  This  object  may  be  accomplished 
by  cautious  doses  of  morpliine  itself,  0.0025-0.005  Gm.  (-iu—ijgT.)  a  day; 
or  by  codeine,  0.005  to  0.01  Gm.  (yV  to  J  gr.);  or  by  heroin,  0.0025  to 
0.005  (^*T-T2  gr-)  twice  a  day.  A  codeine  preparation  (3  per  cent,  codeine 
and  97  per  cent,  extract  of  elecampane  root)  has  recently  been  warmly 
recommended. 

The  saccharated  extract  of  thynms,  2  to  3  spoonfuls  a  day;  hydro- 
chloric phenocoUum  [0.5-3.0  Gm.  (7J-48  gr.)  to  90  per  cent,  of  a 
decoction  of  althaea  and  10.0  of  cherry  syrup,  1  dessertspoonful  every 
two  hours]  and  a  great  many  other  remedies  are  said  to  have  a  specific 
influence  on  whooping-cough. 

Many  observers  claim  to  have  seen  successful  results  from  certain 
physical  curative  methods,  and  a  trial  of  such  under  certain  circum- 
stances can  certainly  be  recommended.  In  this  category  belongs  respi- 
ration in  compressed  air  (in  the  pneumatic  cabinet).  Regular  adminis- 
tration of  tepid  baths  and  carbonic  acid  baths,  will  not  fail  to  influence 
favorably  the  general  condition,  if  not  the  pertussis  itself. 

Within  the  last  few  years  vaccination  has  been  warmly  recommended 
by  many  as  a  prophylactic  and  therapeutic  agency  against  pertussis. 
It  is  claimed  to  bring  about  a  favorable  change  in  the  course  of  whoop- 
ing cough  and  at  times  to  diminish  the  susceptibility  to  infection.  But 
control  experiments  have  proved  vaccination  to  be  inefficient. 

Intubation  (according  to  O'Dwyer)  is  rather  too  energetic  a  method 
to  find  many  advocates.  Yet  it  may  be  beneficial  when  the  course  of 
the  whooping-cough  is  severe. 

The  treatment  of  the  comphcations  of  whooping-cough  depends 
upon  the  nature  of  the  comphcating  disease.  A  prophylactic  measure 
may  be  noted  winch  is  said  to  be  able  to  prevent  the  development  of 
capillary  bronchitis,  namely,  Schultze's  swinging  as  recommended  by 
Raudnitz. 


ACUTE  ARTICULAR  RHEUMATISM 

BY 

Dr.  J.  IBRAHIM,  of  Heidelberg 

TRANSL.\TED    BY 

Dr.  GODFREY   R.  PISEK,  New  York,  X.  Y. 


By  acute  articular  rheumatism,  we  understand  a  febrile  disease  of 
the  joints  characterized  especially  by  transitory  attacks  of  inflamma- 
tion which  pass  intermittent  from  joint  to  joint  and  practically  never 
result  in  suppuration.  To  these  are  added  as  further  characteristics 
the  frequent  involvement  of  the  serous  membranes,  especially  of  the 
endo-  and  pericardium,  as  well  as  the  specific  influence  of  sahcylate 
preparations  on  these  joint  ailments. 

Various  forms  of  polyarthritis  were  formerly  included  in  the  de- 
scription of  acute  articular  rheumatism.  These  diseases,  now  designated 
as  rheumatoid  (Gerhardt)  are  known  to  be  sequela  of  a  variety  of  in- 
fectious diseases,  the  specific  irritants  or  toxins  of  which  have  produced 
them.  Although  they  are  probably  closely  related  to  rheumatic  joint 
diseases,  they  will  be  treated  in  a  special  article,  because  thorough 
scientific  and  therapeutic  knowledge  covering  them  is  of  special  interest. 

Particularly  characteristic  of  acute  articular  rheumatism  in  cliild- 
hood,  is  the  milder  and  shorter  course  of  the  joint  manifestations,  while 
involvement  of  the  heart  and  lasting  injury  to  it  is  more  frequent  than 
in  adults.  Furthermore,  we  notice  especially  in  cliildren  predilection 
for  certain  areas,  for  example  the  joints  of  the  cer\'ical  vertebra^;  as 
well  as  such  comphcations  as  the  formation  of  subcutaneous  nodules 
and  chorea  minor. 

Etiology. — Acute  articular  rheumatism  is  a  disease  found  all  over 
the  world.  Its  appearance  is  commonly  uninfluenced  by  such  factors 
as  natural  phenomena,  character  of  the  soil,  and  underground  water. 
Nevertheless,  frequent  observations  have  shown  that  single  epidemics 
or  a  succession  of  epidemics  occur  from  time  to  time,  although  con- 
tagion by  transmission  has  hardly  in  any  case  been  proven.  It  is  a 
remarkable  fact  that  brothers  and  sisters  can  be  attacked  almost 
simultaneously,  and  judging  from  a  series  of  observations  extending 
over  a  number  of  years,  the  disease  has  been  found  quite  frequently 
to  seek  its  abode  in  certain  houses.  In  a  very  few  cases,  and  even 
these  admit  of  doubt,  transmission  from  the  affected  mother  in  cliild- 
birth  has  been  reported. 

11—31  -181 


482  THE   DISEASES   OF   CHILDREN 

If  we  are  inclined  to  give  some  credence  to  the  statement  that  acute 
articular  rheumatism  is  an  infectious  disease,  probabihty  becomes  a 
certainty  when  we  closely  observe  the  chnical  phenomena,  the  fever, 
the  comphcations  relative  to  the  endo-  and  pericardium,  the  pleura 
and  the  skin. 

A  specific  causative  factor  has,  as  yet,  not  been  found.  For  the 
present  it  must  remain  undecided  whether  in  rheumatic  polyarthritis 
we  have  to  deal  with  a  case  of  attenuated  sepsis  wliich  might  be 
caused  bj'  various  germs  (strepto-,  staphylo-,  or  diplococci)  or  whether 
it  is  a  case  arising  from  an  inherent  exciting  cause. 

Moreover,  there  is  another  question  to  be  determined,  whether 
the  manifestations  in  the  joints  (and  of  the  serous  cavities)  are  the 
result  of  the  causative  agent  itself,  or  of  its  toxin. 

Menzer  asserts  that  the  oral  cavity  is  the  portal  of  entry  (as  do 
also  Singer  and  Meyer)  and  considers  the  inflammation  in  the  joints  and 
serous  cavities  as  reactive  reparative  efforts  of  the  organs  attacked  by 
the  causative  agents  (streptococci). 

Although  the  real  etiological  factor  of  the  disease  is  yet  unknown, 
we  do  know  a  number  of  predisposing  causes,  which  are  co-operative 
in  the  development  of  the  disease.  In  the  first  place  "catching  cold" 
must  be  mentioned,  the  influence  of  wliich,  not  only  in  a  first  attack, 
but  also  in  cases  of  relapse,  can  not  be  doubted.  Very  often  the  onset 
of  the  disease  follows  a  single  severe  exposure  or  wetting,  particularly 
of  the  feet.  Cold  damp  dweUings  play  an  important  part  in  the  devel- 
opment of  infection.  A  constant  influence  of  the  seasons  upon  the  num- 
ber of  cases  has  not  yet  been  proven.  No  doubt  hereditary  influences 
may  here  play  an  important  part.  They  are  absent  in  some  cases.  It 
is  by  no  means  rare  that  in  certain  famihes  rheumatic  diseases  run 
through  several  generations  mth  an  increasing  ratio.  That  a  trauma- 
tism of  the  joints  can  directly  change  to  true  articular  rheumatism,  has 
often  been  observed,  especially  in  children  (Biedert,  Marfan,  Boseck). 
The  sex  of  the  cliildren  seems  to  be  without  noticeable  influence. 

As  to  age,  it  may  be  said  that  the  latter  half  of  childhood  is  by 
far  more  frequently  attacked  by  the  disease  than  the  earher;  in  fact 
its  appearance  is  even  rare  before  the  fifth  year.  We  have  however 
seen  it  come  as  early  as  the  second  or  third  year  of  hfe,  and  even  in  the 
nursing  period  some  well  substantiated  cases  have  been  recorded. 
Even  if  the  disease  has  been  cured,  it  increases  the  predisposition  to 
subsequent  attacks. 

SYMPTOMS  OF  ACUTE  ARTICULAR  RHEUMATISM 
Prodromal    Symptoms.  — Very    frequently    manifestations    of    an 
indefinite  nature  precede  the  outbreak  of  the  disease  itself,  as  languor, 
anorexia,  dragging  pains  in  the  joints,  and  sometimes  even  abdominal 


ACUTE  ARTICULAR  RHEUMATISM  483 

pains.  Catarrhal  or  follicular  angina  appear  as  forerunners  in  a  num- 
ber of  cases;  but  as  far  as  clinical  observations  go  they  do  not  play  such 
an  important  part  as  some  recent  authors  ascribe  to  them.  Often  all 
prodromal  symptoms  are  wanting,  and  the  disease  appears  with  or  with- 
out vomiting  in  its  full  severity. 

Joint  Localization. — The  knee-  or  ankle-joints  arc  those  wliich 
are  primarily  and  most  frequently  attacked.  Often,  however,  it  is  the 
elbows,  wrists  or  shoulders,  wliich  initiate  the  disease.  But  some- 
times the  liip  or  the  cervical  vertebra^,  and  less  frequently  the  joints  of 
the  small  fingers  or  toes,  are  first  attacked.  As  in  the  case  of  adults,  the 
disease  passes  in  its  course  from  joint  to  joint,  either  symmetrically 
or  often  advancing  irregularly  in  its  progress.  More  than  one  joint  is 
always  attacked.  When  new  joints  are  involved,  the  inflammation  in 
those  primarily  affected  as  a  rule  recedes,  but  it  may  persist.  It  is  not 
rare  that  a  joint  may,  in  the  course  of  the  disease,  be  repeatedly  at- 
tacked. The  attacks  in  an  individual  joint  vdW  generally  last  only 
several  hours;  a  duration  of  two  or  three  days  in  childhood  is  the  excep- 
tion. In  the  disease  which  is  characterized  pathologically  and  anatom- 
ically as  a  serous  syno\'itis,  the  joints  involved  are  particularly  painful 
upon  active  and  passive  motion,  and  tender  to  palpation.  Still  the  pains 
are  seldom  so  intense  as  to  cause  complete  immobiUty  of  the  joints. 
An  exception  to  tliis  rule  is  that  in  cliildren  the  frequent  locahzation  in 
the  liip-joints  may  cause  an  intense  degree  of  suffering.  The  objective 
symptoms  of  inflammation  in  children  are  apt  to  be  moderate,  shght  swel- 
ling and  periarticular  oedema  are  often  found,  while  intense  redness  and 
copious  effusions  are  absent  as  a  rule.     Pain  is  often  the  onl}'  symptom. 

The  temperature  does  not  follow  any  definite  type,  still  it  seems 
that  invasion  of  new  joints  or  the  serous  membranes  is  accompanied, 
as  a  rule,  by  a  higher  degree  of  fever;  also  mild  cases  often  begin  with  a 
single  rise  of  temperature,  over  39°  C.  (102°  F.);  in  severer  cases  the 
fever  may  last  several  weeks,  but  this  is  the  exception.  In  general, 
however,  particularly  under  the  constant  influence  of  salicylate  ther- 
apy, the  temperature  returns  after  a  few  days  to  the  normal,  although 
the  rectal  temperature  in  the  evening  may  continue  for  some  time  at 
37.6°-37.9°  C.  (99.6°-100.2°  F.). 

The  general  condition  of  the  patient  in  the  beginning  is  often  con- 
siderably affected.  Children  show  an  unusually  chstressed  countenance; 
the  tongue  is  coated  and  pasty,  the  skin  becomes  remarkably  pale,  such 
palor  is  often  seen  from  the  outset  of  the  disease,  but  especially  if  the  pain 
continues  for  any  length  of  time.  Careful  examination  of  the  blood  does 
not  show  any  special  findings  aside  from  an  insignificant  occasional 
leucocytosis  (Baginsky).  The  marked  tendency  to  profuse  acid  per- 
spiration common  in  adults,  is  frequently  observed  in  children,  although 
in  a  lesser  degree.     The   bowels   are   generally    constipated;    there   is 


484  THE    DISEASES   OF   CHILDREN 

severe,  tormenting  tliirst,  with  anorexia  and  insomnia.  The  general 
condition  depends  upon  the  course  of  the  fever;  in  most  cases  it  im- 
proves by  the  end  of  the  first  week. 

The  duration  of  the  disease,  as  regards  the  joints,  seldom  exceeds  ten 
to  fourteen  days;  often  the  whole  illness  is  over  in  five  to  seven  days.  As 
a  special  pecuharity  of  rheumatism  in  cliildren  it  must  again  be  pointed 
out  that  the  joint  disease  with  its  entire  symptom-complex  may  take  an 
uncommonly  Hght,  almost  afebrile  course  without  seriously  influencing 
the  general  behavior  of  the  patient.  Nevertheless,  these  abortive 
cases  may  be  attended  with  very  severe  heart  comphcations. 

Complications. — As  comphcations  of  acute  articular  rheumatism 
we  here  classify  a  series  of  processes  which  either  positively  or  with 
more  or  less  probabihty  may  be  taken  to  be  notliing  more  than  further 
manifestations  of  the  disease  developing  from  its  irritant  poisons. 
First  in  importance  must  be  mentioned  the  involvement  of  the  heart- 
The  frequency  of  cardiac  comphcations  characterizes  rheumatism  in 
children  as  a  serious  dangerous  disease.  More  than  half  of  all  cases 
leave  beliind  them  permanent  cardiac  defects.  The  heart  remains  nor- 
mally active  during  the  entire  course  of  the  disease  only  in  a  small 
minority  of  the  patients.  This  explains  the  disproportionately  high 
pulse  rate  which  we  see  in  nearly  all  cases,  even  if  chnically  there  can 
be  demonstrated  no  defect  in  the  heart  itself.  In  most  cases  (from  60  to 
80  per  cent.)  verrucose  endocarditis  results,  locahzed  in  the  great  ma- 
jority of  cases  at  the  mitral  valve.  Only  by  careful  and  exact  clinical 
observation  can  we  determine  the  occurrence  of  such  an  unfortunate 
development.  Then  there  appear  recognizable  heart  murmurs,  which 
are  generally  accompanied  by  rise  of  temperature,  irregularities  or  other 
changes  in  the  action  of  the  pulse.  Since  cliildren  under  eight  years  of 
age  seldom  suffer  from  intense  locahzed  pain  and  palpitation,  it  may 
happen  that  children  whose  acute  rheumatism  has  had  an  abortive 
course  are  first  brought  for  medical  treatment  with  signs  of  beginning 
cardiac  insufficiencj'. 

Endocarditis. — Endocarditis  usually  appears  at  about  the  end  of 
the  first  week,  and  often  even  sooner.  But  in  cliildhood  there  are  also 
cases  in  wliich  the  endocarditis  precedes  the  locahzation  in  the  joints. 
Indeed  endocarditis  alone  may  be  discovered,  and  only  later  when 
attacks  of  articular  rheumatism  follow,  does  the  thought  suggest  it- 
self, that  the  heart  disease  may  be  of  a  rheumatic  nature.  In  the  very 
early  period  of  cliildhood,  such  a  course  is  apt  to  occur  quite  frequently 
(Schlossmann).  The  possibihty  that  abortive  articular  rheumatism 
has  existed  must  therefore  always  be  borne  in  mind. 

Pericarditis. — Next  to  endocarditis,  pericarditis  is  an  important 
and  most  fatal  comphcation.  The  involvement  of  the  pericardium  too, 
is  more  frequent  in  children  than  in  adults;   it  manifests  itself  in  about 


ACUTE  ARTICULAR  RHEUMATISM  485 

10  to  20  per  cent,  of  all  cases,  being  nearly  always  associated  with 
endocarditis.  It  is  by  far  the  most  frequent  cause  of  death  in  children 
ha^^ng  rheumatism. 

With  serous  or  even  serofibrinous  pericarditis,  a  case  of  cor  villo- 
sum  may  develop,  or  a  partial  or  complete  obUteration  of  the  peri- 
cardium with  injurious  effect  upon  the  heart  action  may  follow.  And 
it  is  just  this  combination  of  valvular  defects  and  pericardial  adhesions, 
together  with  a  myocarditis  which  is  quite  natural  in  such  cases,  that 
may  bring  about  a  fatal  termination  to  the  disease,  often  so  insidiously 
begun,  after  weeks,  months  or  years  of  suffering. 

Other  Complications. — Pleuritis  of  a  serous  or  serofibrinous 
nature  is  a  compUcation  wliich  is  not  rare  in  chidlhood.  Of  course 
this  occurs  only  in  severe  cases,  and  then  only  in  combination  with 
pericarditis.  It  is  apt  to  be  non-mahgnant,  and  even  a  considerable 
exudate  may  be  readily  absorbed.  Other  still  rarer  complications  in 
childhood  are  bronchitis,  pneumonia,  and  nephritis. 

Angina  and  nose-bleeds  in  the  course  of  articular  rheumatism  are 
often  met  with  in  children.  Occasionally  a  purpuric  rash  appears  in 
the  neighborhood  of  the  affected  joints. 

A  pecuhar  relation  hitherto  unexplained,  exists  between  chorea 
minor  and  the  articular  rheumatism  of  cliildhood.  It  may  precede  the 
joint  disease,  but  in  the  majority  of  cases  it  follows  it.  Like  articular 
rheumatism,  it  is  often  complicated  by  verrucose  endocarditis,  and 
like  the  latter,  chorea  seems,  in  some  cases,  to  take  the  place  of  joint 
attacks.  A  more  complete  description  of  the  phenomena  may  be  found 
in  the  section  on  chorea  minor. 

As  to  the  relation  of  erythema  exudativum  multiforme  and  ery- 
thema nodosum  to  articular  rheumatism  I  must  refer  to  the  respective 
sections  in  this  book.  Certain  eye  affections,  more  or  less  correctly 
classified  as  rheumatic,  e.g.,  iritis,  skleritis,  cannot  be  discussed  here. 

SPECIAL  COURSES  OF  THE   DISE.\SE 

Some  conditions  peculiar  to  cMldhood  may  be  mentioned  here. 
Severe  psychical  and  nervous  derangements,  as  hemi-  and  paraplegia 
and  the  so-called  hijperpyretic  form,  wliich  usually  with  a  temperature 
of  41°-43°  C.  (105.4°-109°  F.)  leads  to  death,  have  been  observed  in 
childhood,  but  only  in  very  rare  cases." 

The  localization  of  the  disease  in  the  joints  of  the  cervical  vertebra 
often  appearing  with  a  rheumatic  torticolhs,  is  not  rare  in  childhood; 
it  is  often  misinterpreted  as  a  purely  muscular  affection,  as  has  been 
emphasized  by  Lannelongue  and  Marfan.  Sometimes  it  may  be  the 
only  seat  of  the  disease,  resisting  all  therapeutic  measures  with  a  per- 
tinacity unusual  in  childhood  and  may  in  this  way  lay  the  foundation 
for  a  subsequent  chronic  arthritis. 


486  THE   DISEASES   OF   CHILDREN 

A  form  of  rheumatism  almost  peculiar  to  cluldhood  is  nodular 
rheumatism,  which  was  first  described  by  Meynet  in  1875  and  obtained 
greater  publicity  through  the  writings  of  Rehn  and  Hirschsprung. 
Over  40  cases  have  been  reported,  mostly  by  English  authors.  It 
usually  occurs  in  the  course  of  an  attack  of  acute  articular  rheuma- 
tism, or  sometimes  during  a  relapse,  generally  in  the  third  week  or  later 
from  the  onset  of  the  joint  symptoms.  Subcutaneous  nodules  appear 
under  the  unchanged  skin,  developing  very  rapidly,  often  over  night. 
They  are  more  or  less  symmetrically  located  in  the  \'icinity  of  the  joints 
and  along  the  tendons  but  may  be  in  distant  parts  of  the  body,  e.g., 
on  the  bones  of  the  skull.  They  are  the  size  of  a  pin-head,  or  even  a  nut, 
and  only  rarely  are  firmly  attached  to  the  periosteum  or  tendons.  They 
are  rather  sensitive  to  pressure  and  consist  of  fibrous,  or  partly  fibro- 
cartilaginous tissue  (Henoch,  Hirschsprung,  Barlow).  Drewitt  considers 
them  analogous  to  the  nodules  of  rheumatic  endocarditis.  Their  num- 
ber varies  from  one  to  more  than  fifty.  Tlie  nodules  generally  soon 
disappear.  Rarely  their  re-absorption  extends  over  weeks  or  months 
in  the  severer  cases  of  rheumatism,  wliich  are  almost  always  compli- 
cated with  endo-  or  pericarditis.  Some  cases  with  chorea  were  repeat- 
edly observed  and  one  case  had  also  erythema  multiforme. 

Course  and  Prognosis. — Most  cases  of  polyarthritis  in  children 
get  well  quickly  and  completely;  on  the  other  hand,  it  may  be  with  a 
defect  of  the  cardiac  valves.  Relapses  are  frequent  in  cliildren,  and 
it  is  not  uncommon  to  have  endocarditis  develop  during  a  second  or 
third  attack,  if  a  pre\'iously  existing  lesion  should  grow  worse.  Death 
occurs  almost  without  exception  from  severe  cardiac  compUcations; 
particularly  pericarditis,  but  occasionally  from  multiple  emboli.  In 
rare  cases  swelling  and  stiffness  may  remain  in  some  of  the  joints.  Re- 
peated relapses  may  cause  a  transition  into  the  chronic  articular  variety. 

So  far  as  hfe  is  concerned,  the  prognosis  is  more  favorable  in  chil- 
dren than  in  adults.  It  is,  however,  entirely  dependent  upon  the  severity 
of  the  heart  complications. 

Diagnosis. — The  diagnosis  of  acute  articular  rheumatism  is  usually 
readily  made.  Nevertheless  there  are  cases  in  wliich  difficulties  occur, 
and  where  a  false  diagnosis  might  be  fatal.  This  applies  especially  to 
those  forms  of  rheumatic  arthritis  in  wliich  prompt  surgical  interfer- 
ence is  indicated,  as  in  the  cases  of  septic  and  pneumococcic  arthritis. 
At  any  rate  the  possibility  of  the  presence  of  a  rheumatoid  condition 
must  be  considered,  and  of  course  one  must  exclude  scarlet  fever  and 
gonorrha^a. 

The  characteristic  symptoms  of  pneumococcic  arthritis  will  be 
described  below.  The  diagnosis  of  rheumatic  arthritis  is  based  in  gen- 
eral on  the  migratory  attacks  in  the  joints,  the  character  of  the  in- 
flammatory  process,  the   polyarticular   localization  and  the  beneficial 


ACUTE  ARTICULAR  RHEUMATISM  487 

influence  of  salicylate  therapy.  Endocarditis,  if  present,  it  is  true, 
does  furnish  in  the  case  of  rheumatoid  conditions,  a  most  valuable 
and  an  indisputable  element.  Cases  of  hereditary  syphilitic  joint  affec- 
tions deserve  special  attention.  They  follow  a  subacute  course  and, 
attacking  the  two  knee-joints  symmetrically,  are  often  incorrectlj'  diag- 
nosticated. Further  details  regarding  these  cases  will  be  given  on 
page  492.  In  a  case  of  syphilitic  osteochondritis,  only  a  careless  exami- 
nation can  possibly  lead  to  a  wrong  diagnosis. 

From  a  differential  standpoint  it  may  be  said  that  an  atypical  at- 
tack of  articular  rheumatism,  beginning  with  severe  pain  in  the  hips, 
may  at  first  impress  one  as  a  severe  case  of  suppurative  coxitis.  In 
one  case  under  my  observation  the  diagnosis  of  sciatica  was  also 
erroneously  made.  Sometimes  the  distinction  between  spinal  caries 
or  beginning  retropharyngeal  abscess  and  rheumatism  of  the  cer^^cal 
portion  of  the  vertebral  columii  may  create  difficulties.  The  presence 
of  osteomyelitis,  too,  may  occasionally  appear  probable.  An  accurate 
local  examination  as  well  as  the  course  of  the  disease  will  readily  clear 
up  the  diagnosis. 

In  Httle  children  and  infants  who,  by  the  way,  are  more  frequently 
subjected  to  all  kinds  of  rheumatoids  than  to  true  acute  articular  rheu- 
matism, infantile  scurvy  must  occa.sionally  be  differentiated  from  poly- 
arthritis. Careful  examination  of  the  gums  and  urine  and  localization  of 
the  pain  in  the  limbs  mil  prevent  a  wrong  diagnosis.  Articular  swelHng, 
which  sometimes  occurs  after  the  injections  of  a  curative  serum,  is  di- 
rectly traced  to  its  cause  by  a  knowledge  of  the  history.  It  must  again  be 
stated  that  an  abortive  course  of  articular  rheumatism  is  often  called 
gromng  pains,  and  onh*  later  on,  because  of  an  endocarditis  or  chorea 
developing,  is  it  correctly  diagnosticated. 

Prophylaxis  is  especially  important  in  children  who  either  have 
already  had  an  attack  of  articular  rheumatism  or  chorea,  or  who  are  de- 
scendants of  rheumatic  families.  Dry  and  healthy  habitations  and  avoid- 
ance of  exposure  to  cold  or  wetting  are  very  essential  factors.  Often 
the  wearing  of  woolen  or  merino  undergarments  is  to  be  recommended. 
A  hardening  process  by  means  of  hydrotherapy  carefully  begun  under 
systematic  medical  guidance  is  particularly  beneficial. 

Treatment. — Two  conchtions  must  always  be  complied  \\'ith  :  rest 
in  bed  until  all  manifestations  cease  and  a  constant  regulation  of  the 
temperature  of  the  room  at  14°-15°  R.  (62°-65°  F.).  Every  opportunity 
to  acquire  a  cold  subjects  the  patient  to  the  possibihtj*  of  a  relapse 
and  a  prolongation  of  liis  sufferings.  During  nursing,  and  the  chang- 
ing of  hnen,  etc.  tliis  possibihty  must  be  carefully  watched,  as  the 
profuse  perspirations  which  are  present  in  the  disease,  or  possibly  also 
the  salicylate  treatment,  may  enhance  the  danger  of  chills  and  colds. 
The  bed  should  stand  in  the  most  protected  part  of  the  room  and  above 


488  THE   DISEASES   OF   CHILDREN 

all  not  close  to  a  cold  wall.  Woolen  garments,  even  when  in  bed,  are 
often  of  advantage.  Warm  but  not  too  heavy  bed  covering  is  important, 
and  should  be  the  special  care  of  the  nurse,  particularly  \vith  the  younger 
children,  who  are  apt  to  uncover  themselves. 

Diet. — At  the  height  of  the  fever:  bouillon,  milk,  wheat  bread, 
barley  or  oatmeal  broth,  or  pap;  at  the  same  time  plenty  of  fluid  in  the 
form  of  lemonade  and  soda  water.  When  the  temperature  falls,  vege- 
tables, meat  and  eggs  are  permitted. 

SaUcyUc  acid  is  specifically  effective  for  controlhng  the  fever  and 
the  articular  phenomena,  and  is  best  administered  as  salicylate  of  soda 
or  aspirin. 

Effective  action  against  the  invasion  or  severity  of  the  heart  compli- 
cations is  unfortunately  not  assured  either  by  the  above  or  by  any 
other  measure.  At  the  outset  not  too  minute  doses  should  be  given, 
to  smaller  cliildren  0.25  Gm.  (about  5  gr.),  to  larger  cMldren  0.5-0.8 
Gm.  (7i-12  gr.)  three  to  four  times  daily,  as  a  powder  or  in  aqueous 
solution.  When,  after  the  lapse  of  1  to  2  days,  the  symptoms  have 
vanished,  the  daily  dose  of  1  Gm.  (15  gr.)  for  older  children,  and  0.5 
Gm.  (7J  gr.)  for  younger  ones  may  be  persisted  in  for  6-8  days  more, 
in  order  to  avert  another  attack  in  the  joints. 

R     .\spirin 0.5 gr.  vii 

D.  t.  d.  vi. 
Sig. — Tliree  times  daily — \-l  powder. 

R     Salic,  sod 5.0-10.0..  .3iss-iiss 

Tinct.  aurantii  cort 2.0 oss 

Aq 100.0 5iii 

M.  D.  S. — One  half  to  one  teaspoonful  tliree  times  a  day. 

Tliis  medication  is  followed  l)y  more  or  less  profuse  perspiration. 
Other  secondary  effects  hardly  occur  if  the  above-mentioned  doses  are 
adhered  to,  excepting  ringing  in  the  ears,  which,  however,  is  not  an 
indication  to  discontinue  the  saUcylate  preparations. 

Heubner  observed  no.se  bleeding  in  a  few  cases,  and  we  ha^■c  seen 
albuminuria  appear  twice,  wliich  slowly  vanished  after  discontinuing 
the  sahcylates.  In  case  sahcylic  acid  or  aspirin  prove  ineffective,  one 
of  their  substitutes  may  be  successful,  as  phenacetin,  antipyrin,  salol, 
sahpyrin  and  lactophenin,  in  such  doses  as  are  appropriate  for  children. 
In  these  cases,  which  however,  are  rare,  even  the  above-mentioned  rem- 
edies may  fail,  and  it  is  then  always  well  to  ijeconsider  the  diagnosis. 
We  may  often  be  forced  to  conclude  that  we  have  a  case  of  rheuma- 
toid before  us,  or  else  one  of  those  sad  cases  with  a  tendency  to  change 
into  the  severe  chronic  form,  the  treatment  of  which  will  be  described 
on  page  498. 

In  connection  with  the  internal,  a  local  treatment  may  be  required 
if  the  pains  are  .'severe.     Simply  enveloping    ^\^th    cotton  wadding  or 


ACUTE  ARTICULAR  RHEUMATISM  489 

immobilizing  in  splints  is  often  found  to  give  great  relief.  By  means  of 
■'mesotan"  local  salicylic  treatment  can  be  had  in  place  of  the  internal 
salicyHc  doses. 

R     Mesotan 20.0 ov 

Ol.Ricini 30.0 s'l 

M.  D.  S. — 3-5  Gm.  (h  to  one  teaspoonful)  painted  over  tlie  joints.  (One  must  be 
on  the  lookout,  however,  for  dermatitis.) 

If  after  the  acute  stage  has  passed,  swelUng  or  stiffness  of  individ- 
ual joints  remains,  inunctions  with  ichthyol-vaseline  (10  per  cent.)  or 
Priessnitz  compresses  followed  by  massage  ma)^  render  good  service. 
If  there  is  the  least  suspicion  of  ensuing  endocarditis,  the  measure  most 
indicated  and  most  important,  though  very  difficult  to  carry  out  \vith 
children,  is  absolute  bodily  rest;  with  the  customary  application  of 
icebags,  it  is  important  to  avoid  needless  uncovering  of  the  patient,  or 
accidental  wetting,  lest  a  relapse  of  the  joint  symptoms  be  caused.  AVith 
regard  to  an  estabUshed  endocarditis,  pericarditis,  etc.,  we  refer  to  their 
relative  sections. 

Rheuniatis}y}us  nodosus  requires  no  special  method  of  treatment.  For 
a  rheumatic  involvement  of  the  cervical  portion  of  the  vertebral  col- 
umn, it  is  advisable,  if  salicylic  therapy  does  not  bring  speedy  recovery, 
to  resort  to  the  apphcations  of  warm  poultices  and  carefully  conducted 
massage.  For  severe  cases,  some  new  methods  of  treatment  which 
have  been  recently  proposed  may  be  briefly  mentioned  here:  The 
intravenous  (Mendel)  or  intra-articular  (Bouchard)  applications  of 
saUcylic  acid;  scarification  and  cauterization  of  the  tonsils  (Giirich); 
the  use  of  streptococcic  serum  (Meyer,  Menzer).  Menzer  expects, 
through  his  serum,  to  be  able  to  reduce  the  frequency  of  cardiac  involve- 
ment. The  value  of  these  methods  is  up  to  the  present  time,  as  far  as 
adults  are  concerned,  open  to  discussion;  their  general  appUcation  in  the 
case  of  children  would  be  therefore  much  less  advisable. 

SPECIFIC  ARTHRITIDES 
RHEUMATOIDS — PSEUDORHEU.MATISM 

Multiple  joint  infections  occur  in  the  course  of,  or  as  a  sequel  to, 
many  acute  infectious  diseases.  In  some  cases  the  specific  micro- 
organism can  be  found  in  the  secretions  of  the  joint.  A  consideration  of 
the  several  characteristic  forms  is  of  importance  from  a  diagnostic  and 
therapeutic  standpoint.  These  infections  are  not  uncommon  in  cliild- 
hood  and  infancy.  Among  them  may  be  mentioned  typhoid,  diph- 
theria, cerebrospinal  meningitis,  German  measles,  influenza,  etc.  The 
most  frequent  and  distinctive  forms  are  described  below. 

SEPTIC   POLY.\RTHRITIS 

I  wish  to  refer  here  only  briefly  to  suppurative  inflammatory  joints, 
associated  with  general  sepsis.  They  are  to  be  found  in  all  periods  of 
childhood,  especially  in  nursHngs,  where  a  puerperal  or  umbilical  infec- 


490  THE   DISEASES   OF   CHILDREN 

tion  may  be  its  basis.  AVe  may  also  call  attention  to  (secondary)  strepto- 
coccic sepsis  following  scarh^t  fever,  which  frequently  produces  purulent 
arthritis.  The  joint  symptoms  in  themselves  do  not  manifest  the  pres- 
ence of  sepsis.  In  doubtful  cases  the  high  leucocytosis  will  preclude 
rheumatic  polyarthritis.  Diagnosis  by  exploratory  puncture  and  a  mi- 
croscopic examination  of  the  pus  should  be  made  immediately,  since  a 
prompt  incision  may  effect  a  cure,  even  in  nursUngs  (conip.  Schlossmann). 

GONORRHCEAL    ARTHRITIS 

In  comparison  to  gonorrhceal  vulvitis  wliich,  according  to  our 
experience,  is  rather  frequent,  gonorrhceal  joint  inflammation  in  chil- 
dren is  decidedly  rare.  It  appears  more  frequently  as  a  sequel  to  puru- 
lent ophthalmia;  and  to  gonorrha'al  urethritis  in  boys.  Arthritis 
appears  most  generally  in  the  secontl  or  third  week  subsequent  to  the 
local  attack.  It  is  especially  apt  to  attack  the  knee-joints.  Among 
others  the  temporomaxillary  and  sternal  joints  may  likewise  be  at- 
tacked. The  articulations  are  usually  very  painful,  Irighly  reddened  and 
swollen.  Puncture  shows  seropurulent  fluid,  in  which  the  presence  of 
gonococci  of  typical  stratification  can  be  found  under  the  microscope 
or  even  by  culture.  High  irregular  fever  usually  accompanies  the 
affection.  The  course  of  the  disease  is  with  cliildren  and  infants  in  par- 
ticular usually  a  rapid  one,  seldom  exceeding  two  or  three  weeks,  and 
recovery,  even  functional,  is  as  a  rule  complete.  CompUcations  with 
tendovaginitis  (Seiffert)  endocarditis  (Chiaso  and  Isnardi,  Schloss- 
mann, Hermann)  and  pleuritis  (Mazza,  Bordoni-Uffreduzzi,  Chiaso  and 
Isnardi)  occur,  but  even  these  affections  appear  in  children  as  non- 
malignant  and  curative.  A  combination  of  skin- metastases  in  the 
form  of  vesicles  and  papules  is  described  by  Paulsen,  with  this  Hermann 
mentions  gonorrhceal  erythema  exudativum  and  nodosum;  Cassal  saw 
an  abscess  of  the  skin  with  gonorrhceal  suppuration  in  a  child  eleven 
years  old  suffering  from  multiple  gonorrhceal  arthritis. 

The  diagnosis  is  easy  if  we  call  to  mind  the  possibihty  of  the  gonor- 
rhceal nature  of  the  joint  affections.  In  no  case  of  polyarthritis  must 
the  examination  of  the  conjunctivae,  the  vulva,  or  the  male  urethra,  be 
neglected.  Features  distinguishing  it  from  acute  articular  rheuma- 
tism are  the  symptoms  of  an  inflammation  usually  of  greater  intensity, 
a  longer  duration  of  the  locahzation  in  single  joints,  also  relatively  the 
monarticular  locahzation,  the  rarer  compUcation  of  endocarditis,  and 
finally  the  poor  results  of  saHcylate  therapy.  In  doubtful  cases  the 
proof  of  the  presence  of  gonococci  in  the  joint  effusion  (which  by  the 
way  is  not  always  successful)  must  decide. 

The  treatment  brings  results.  Enveloping  with  cotton  wadding 
and  fixation  of  the  joints  generally  suffices.  With  cliildren  beyond  the 
nursing  age  a  trial  with  sodium  saUcylate  is  at  least  advisable. 


ACUTE  ARTICULAR  RHEUMATISM  491 

Hermann  records  a  prompt  result  after  an  intravenous  injection 
of  collargol  [3.0  c.c.  ("i  45)  of  a  2  per  cent,  solution  for  a  fourteen-year-old 
boy].  In  severe  cases  it  would  be  well  to  take  this  method  of  treat- 
ment into  consideration.  Exceptionally  it  may  become  necessary  to 
resort  to  arthrotomy  (Paulsen).  Of  especial  importance  is  a  thorough 
removal  of  the  source  of  gonococcus  infection. 

SCARLATINAL    POLYARTHRITIS 

Inflammation  of  the  joints  occasionally  develops  in  the  course  of 
scarlet  fever,  wliich  maj'  greatly  resemble  acute  articular  rheumatism. 
Generally  it  appears  the  second  or  third  week  of  the  disease,  or  pos- 
sibly sooner,  and  it  may  completely  vanish  in  6  to  10  days.  Most  fre- 
quently the  wrist-joints  are  affected  symmetrically,  then  those  of  the 
shoulders,  knees  and  feet.  As  a  sequel  to  scarlet  fever,  endocarditis 
and  affections  of  the  joints  may  be  combined.  Even  without  salicylate 
treatment  these  cases  of  arthritis  are  apt  to  yield  to  envelopment 
^^■ith  cotton  wadding  and  immobilization.  This  differs  from  the  puru- 
lent joint  inflammations  which  occur  in  scarlet  fever  as  a  result  of 
streptococcic  sepsis,  and  which  require  surgical  treatment. 

PNEUMOCOCCIC    ARTHRITIS 

Pneumococcic  arthritis  wliich  has  only  recently  received  proper  at- 
tention o;\'ing  to  the  labors  of  Pfisterer  and  Herzog,  deserves  special 
mention  in  these  pages,  since  it  is  apt  to  occur  in  childhood,  particularly 
in  the  first  and  second  years  of  life.  Bronchitis  or  pneumonia  may  or 
may  not  precede  the  arthritis.  The  pharynx  and  middle  ear  may  be 
considered  as  portals  of  entrance.  Now  and  then  pneumonia  follows 
the  joint  disease. 

The  pathology  is  one  of  suppurative  effusion,  with  a  copious  infil- 
tration of  the  capsule,  which  like  the  pus  is  permeated  with  diplo- 
cocci  positive  to  Gram's  stain,  while  the  cartilage  shows  little  or  no 
noticeable  change.  The  affection  is  usually  monarticular,  sometimes 
oUgarticular.  The  most  frequently  involved  areas  are  the  shoulder, 
knee,  or  hip.  It  is  rare  to  have  a  true  septic  course  in  wliich  many 
joints  participate  and  lead  to  death.  Comphcations  with  meningitis, 
empyema  and  peritonitis  often  occur  (Hagenbach,  Romheld).  Epiphy- 
seal osteomj-ehtis  may  exist  at  the  same  time  and  perhaps  be  taken  as 
the  primary  cause  of  the  joint  suppuration  (Herzog). 

Diagnostic  Signs. — Characteristic  symptoms  are  great  swelling 
and  widespread  inflammatory  oedema,  with  simultaneous  pallor  of  the 
skin.  Notwithstanding  the  severe  local  manifestations  and  the  liigh 
fever  the  general  physical  condition  and  sleep  are  proportionately  only 
slightly  impaired. 

The   differential  diagnosis   from  articular  rheumatism   and   tuber- 


492  THE   DISEASES   OF   CHILDREN 

culous  coxitis,  requires  no  discussion;  however,  there  is  greater  proba- 
bihty  that  pneumococcus  arthritis  will  be  confounded  with  gonorrhoeal 
arthritis.  Aside  from  the  primary  sources  of  infection,  wliich  can  be 
etiologically  proven,  it  is  necessary  in  all  suspicious  cases  for  the  verifi- 
cation of  the  diagnosis  in  pneumococcic  arthritis  to  make  as  soon  as 
possible  an  exploratory  puncture.  The  lancet  shaped  Gram-positive 
capsulated  diplococci  are  easily  identified  under  the  microscope.  Cul- 
ture and  animal  inoculation  may  be  of  service  for  positive  identification. 

The  prognosis  is  unfavorable  if  the  arthritis  is  only  a  local  manifes- 
tation of  a  severe  general  infection;  otherwise  it  depends  essentially 
upon  a  timely  diagnosis  and  incision.  Thick  creamy  pus  gives  a  better 
outlook  than  a  tliin  pus.  The  curative  result  is,  as  a  rule,  functionally 
perfect. 

As  far  as  therapeutics  is  concerned,  only  the  earhest  and  sufficient 
incision  is  to  be  considered.  Expectant  treatment  or  puncture  are 
positively  to  be  rejected. 

HEREDITARY    SYPHILITIC    ARTHRITIS 

As  a  consequence  of  hereditary  syphiHs,  arthritis  appears  as  a  be- 
lated manifestation  in  children  who  have  not  been  specifically  treated. 
The  recognition  of  these  cases,  for  which  we  are  principally  indebted  to 
ophthalmologists,  is  all  the  more  important  because  antisyphilitic 
treatment  (specifically  with  iodide)  generally  results  in  a  complete  and 
rapid  cure.  Less  frequently  in  cliildren  under  five  (more  frequently  in 
those  between  six  and  ten  years)  there  arises  without  any  traumatism, 
either  subacutely  or  chronically  an  effusion  into  the  joints,  which  may 
become  very  copious,  causing  at  the  same  time  surprisingly  little  sub- 
jective disturbance.  Puncture  shows  serofibrinous  fluid.  Tempera- 
ture is  normal  or  subnormal.  Cases  of  severe  inflammation,  however, 
accompanied  by  liigh  fever  also  accur  (Bosse).  The  knee-joints  are 
most  frequently  attacked,  and  the  disease  almost  always  affects  both 
of  them  in  the  course  of  time.  Therefore,  every  case  of  gonitis  (in- 
flammation of  the  knee-joints)  beginning  in  the  manner  described  (and 
especially  if  the  second  knee  begins  to  be  involved  in  the  same  way),  will 
cause  us  to  suspect  .secondary  sypliilis.  It  will  then  be  necessary  to 
explore  the  family  history  of  the  child  as  well  as  its  physical  condition. 

Should  parenchymatous  keratitis  be  present  along  mth  the  artic- 
ular affection,  it  will  more  easily  verify  the  diagnosis,  unless  the  same 
conclusion  has  been  reached  by  treatment  for  sypliilis  previously  em- 
ployed. It  is  usual  for  hereditary  syphihtic  arthritis  to  be  combined 
\\-ith  parenchymatous  keratitis.  The  arthritis  precedes  the  eye  affec- 
rion  almost  always  by  months  or  years. 

Bosse  found  arthritis  of  an  hereditary  syphihtic  nature  in  37  per 
cent.,  von  Hippel  in  56  per  cent,  of  all  cases  of  parenchymatous  keratitis. 


ACUTE  ARTICULAR  RHEUMATISM 


493 


The  treatment  is  dependent  on  the  diagnosis.  The  specific  treat- 
ment is  generally  successful  even  in  cases  accompanied  with  high  fever 
and  inflammatory  signs  which  appear  to  require  surgical  procedure. 


Fig.  110. 


CHRONIC  ARTICULAR  RHEUMATISM 

By  chronic  articular  rheumatism,  we  understand  a  series  of  types 
of  diseases,  etiologically  and  clinically  not  quite  aUke;  for  the  present, 
a  strict  classification  does  not  appear  advisable.  The  cases,  taken  all 
in  all,  are  rare.  (About  one  hundred  have 
been  reported  in  the  Uterature.)  In  some 
countries,  e.g.,  England,  they  seem  to  be 
more  frequent.  In  childhood,  too,  we  may 
distinguish  two  different  types.  Individual 
cases  of  course  may  present  various  devi- 
ations. 

1.    CASES    COMPLICATING    ACUTE 
ARTICULAR    RHEUMATISM 

Group  (a).  Those  gradually  arising  in 
the  course  of  a  greater  number  of  single 
acute  attacks  (secondary  chronic  arthritis), 
in  which  at  first  only  slight  joint  distur- 
bances remain,  but  become  worse  with  every 
new  attack  and  spread  to  other  joints. 
This  is  in  general  the  mildest  form  so  far 
as  prognosis  is  concerned  (Fig.  110). 

Group  (b).  Developing  directly  from 
the  first  acute  attack,  without  the  occur- 
rence of  even  a  temporary  return  of  symp- 
toms. From  the  very  beginning  these  cases 
are  characterized  by  their  unusual  localiza- 
tion or  peculiar  course  (Heubner)  in  that 
the  disease  attacks  preferably  the  small 
finger-joints  with  co-participation  of  the 
temporomaxillary  and  sternocla\Tcular 
joints,  the  symphyses,  etc.  Disease  of  the 
cervical  portion  of  the  vertebral  column 
especially  may  initiate  such  cases;  and 
furthermore  the  salicylate  often  proves  to 
be   partly   or   altogether  ineffectual    (Figs. 

Ill  and  112).  These  cases  of  the  first  group  often  appear  in  com- 
bination with  endo-  and  pericarditis  and  they  show,  starting  from 
the  large  joints,  a  centrifugal  progression  in  the  involvement  of  the 
joints.      Here  might  be   added   those   rare   cases   of    chronic    articular 


Secondary  chronic  arthritis  follow- 
ing acute  articular  rheumatism.  Girl 
nine  years  old.  Acute  articular  rheu- 
matism. At  seven  and  a  half  years 
joint  residuals,  originally  only  in  the 
joints  of  hands  and  feet.  Progressive 
participation  of  the  large  and  all  the 
small  joints  of  the  extremities,  and  of 
the  vertebral  column,  partly  accom- 
panied by  attacks  of  pain.  Insuffi- 
ciency and  stenosis  of  the  mitral  and 
tricuspid  valves.  Epileptic  attacks; 
chronic  pachymeningitis,  encephalitis 
(of   a    rheumatic    origin?)  Death. 


494 


THE   DISEASES   OF   CHILDREN 


rheumatism    which    pursue    a  similar   course  as  sequels   to   the   acute 
infectious   diseases   (influenza,  scarlet  fever,  measles,  see  Fig.  113). 

2.    CASES    OF    PRIMARY    CHRONIC    ARTHRITIS 

The  disease  begins  subacutely  or  chronically,  without  or  attended 
by  more  or  less  severe  attacks  of  pain,  and  may  run  a  subfebrile  course. 
Centripetal    progression    starts    from    the    small    finger-  or   toe-joints. 
Sometimes  the  knee  and  elbow  may  have   been  previously  attacked. 
F,,;.  111.  Endocarditis  is   only 

r  ^        exceptionally  noticed 

(see     Figs.    114    and 
115). 

Of  sixty-six  cases 
I  found  the  beginning 
of  the  disease  men- 
tioned in  twenty-sev- 
en individuals,  in  the 
first  to  the  fifth  year 
of  Ufe  (two  of  them 
in  the  first,  eight  in 
the  second,  ten  in  the 
third,  fourteen  in  the 
fourth)  twenty-two  in 
the  six  to  the  tenth, 
seven  in  the  eleventh 
to  the  fourteenth 
year. 

The  articular 
changes  themselves 
cannot  be  strictly 
distinguished  clini- 
cally or  anatomi- 
cally in  either  type. 
In  general  the  re 
is  at  first  an  effusion  or  a  doughy  swelhng;  later  on  more  marked  changes 
in  the  capsular  ligaments  and  the  surrounding  soft  parts  (villous 
proUferation,  contraction  of  ligaments)  in  consequence  of  which  there 
is  a  decided  impediment  to  motion,  finally  positive  contractions  and 
immobilization,  resulting  in  fibrous  adhesions  and  production  of  anky- 
losis together  wdth  erosion  and  connective  tissue  transformation  of  the 
articular  cartilages.  Further,  and  usually  later  development  shows  par- 
ticipation of  the  bones,  changing  them  into  the  types  wliich  cannot  be 
distinguished  from  the  so-called  deformative  joint  processes.  Sepa- 
rations of  the  epiphyses  likewise  occur  (Spitzy). 


Secondary  chronic  artliritis  following  acute  articular  rheumatism  in 
the  second  year  of  life.  Girl  seven  years  old.  Swelling  and  immobility 
of  all  the  joints  of  the  e.'wtremities.  with  participation  of  the  dorsal 
and  cervical  portions  of  the  vertebrae.  High  grade  muscular  atrophy: 
endocarditis. 


ACUTE  ARTICULAR  RHEUMATISM 


495 


Fig.  112 


From  a  clinical  standpoint,  the  nodular  swelling  of  the  finger-joints  is 
particularly  striking  (Figs.  112, 114, 115).  The  skin  from  time  to  time  as- 
sumes a  glazy  inflamed  appearance.  Deviations  of  the  fingers  or  toes  to  the 
ulnar  side  do  not  seem  to  occur  in  oliildhood,  hut  another  kind  arc  seen. 

Certain  locaUzafions,  quite  freiiuent  in  the  chronic  forms,  are  apt 
to  put  the  poor  suffering  patient  into  a  most  lamentable  state.  This 
is  also  the  case  where  the  vertebral  column  is  affected,  particularly 
the  cervical  portion, 
and  sometimes  in  the 
temporomaxillary  and 
costal  articulations. 

Fig.  Ill  represents 
a  girl  in  the  pagoda  at- 
titude, with  almost  com- 
plete immobilization; 
only  movements  of  the 
knees  and  shght  motion 
of  the  arms  were  pos- 
sible, the  hips  and  the 
head  were  nearly  im- 
movable. She  could  he 
only  on  her  side. 

The  symmetrical 
attack  of  both  halves 
of  the  body  is  ex- 
tremely persistent  and 
this  gave  rise  to  the 
opinion  that  the  entire 
affection  is  to  be  re- 
garded as  a  tropho- 
neurosis. In  some 
cases,  however,  this 
symmetry  is  not  well 
marked.  Atmospheric 
changes  may  bring  on  an 
aggravation  of  pain, 
passive  motion  some- 
times   elicits    marked 

crepitation,  particularly  if  the  immobihty  in  the  stiffened  joints  is  dimin- 
ishing. Extreme  atrophy  of  the  muscles  surrounding  the  joint  is  very 
noticeable  in  all  severe  cases,  produced  partially  by  inacti\'ity,  par- 
tially through  reflex  tropho-neurotic  influences  (Hoffa). 

The  extremities  assume,  with,  the  tliickening  in  the  joints,  quite  a 
characteristic  appearance  (see  Fig.  111). 


Secondary  chronic  arthritis  following  acute  articular  rheumatiam. 
The  same  child  as  in  Fig.  111.  Swelling  of  the  wrist-joint,  back 
of  tlie  hand  and  all  the  small  finger-joints. 


496 


THE    DISEASES   OF   CHILDREN 


The  diminution  of  tlie  bone  at  the  diaphysis  is  in  part  due  to  a 
higli  grade  concentric  atrophy  of  the  bones  themselves  (Johannessen, 
Kienboclv,  Reiner).  The  X-ray  pictures  show  a  continuous  inter- 
stitial atrophy  of  the  bones  involved.  An  unusual  diagnosis  by 
means  of  the  X-rays  is  reported  by  Reiner,  namely,  a  corroded  or  spht 
appearance  of  the  badly  swollen  ejjiphyses  of  the  phalanges  of  the 
fingers. 

The  children  for  the  most  part  show  a  moist  ana-mic  skin,  once  in  a 

while,  profuse  perspiration  with  a  mihtary  eruption,  especially  in  pri- 

Fir;  n:*  mary  cases,  followed   in  one  of   my  cases  by 

furunculosis.     Generally  there  is  no  fever,  but 

it  is  more  apt  to  occur  in  the  primary  cases. 

Endocarditis  with  valvular  lesions  is 
found  only  in  cases  of  the  first  group  (see 
above). 

Rare  occurrences  are  abnormal  lengthen- 
ing and  tliickening  of  the  large  toe  (Johan- 
nessen, Spitzy),  diminution  and  lessening  of 
the  lower  jaw  (Diamantberger),  prevention 
of  the  growth  and  development  of  all  the 
extreniities  (Hoppe-Seyler)  and  exophthal- 
mos (Diamantberger).  Only  one  case  belong- 
ing to  the  first  type,  that  of  Henoch,  exhibited 
rheumatic  nodules. 

A  specific  type  not  yet  explained  as  to 
its  nosological  value,  is  represented  by  Still's 
disease.  Here  is  found  an  almost  painless 
chronic  tlrickening  and  stiffening  of  the  joints 
of  Uttle  children,  accompanied  by  fever,  either 
continuous  or  periodical.  The  disease,  begin- 
ning with  the  knee-joint,  wrists,  or  the  cer- 
vical portion  of  the  vertebral  column,  gradu- 
ally attacks  the  ankles,  elbows  and  fingers, 
without  leading  to  any  destructive  articular  transformations.  There  is 
no  endocarditis,  but  at  the  same  time  pericardial  adhesions  have  been 
noticed.  The  most  conspicuous  manifestation  is  the  hypertrophy  of  the 
spleen  and  the  multiple  sweUing  of  the  lymphatic  glands.  It  yet 
remains  an  open  question,  whether  or  not  these  cases  are  possibly  due 
to  tuberculosis  or  to  chronic  sepsis. 

Course  and  Termination. — The  course  of  the  affliction  is  chronic 
in  the  extreme,  and  may  extend  into  years  and  eventually  decades.  At 
any  rate  the  affection  advances  in  cliildren  with  greater  rapidity  than  in 
adults.  Long  intermissions  occur.  Recovery  or  at  least  considerable 
improvement  is  seen  especially  in  cases  of  the  first  group.     Improve- 


Secondary  clironic  arthritis  fol- 
lowing measles.  Boy  four  years 
old.  Hand-,  knee- and  ankle-joints 
swollen.  Fing:er-joint  unaffected. 
(Spitzy.) 


ACUTE  ARTICULAR  RHEUMATISM 


4!)7 


ment  is  found  also  in  very  rare  cases,  of  course,  in  children  of  (lie  second 
group.  Death  is  either  the  result  of  general  exhaustion  or  of  a  second- 
ary tuberculosis. 

Diagnosis. — The  differential  diagnosis  should  present  no  difficul- 
ties except  to  exclude  sypliilis  and  tuberculosis.  Regarding  the  former 
we  refer  to  its  proper  section.  Multiple  fungous  joint  disease  may  pro- 
duce an  appearance  which  is  very  similar  to  clironic  rheumatism;  liere 
however,    tuberculous    di.s-  fig.  lu. 

ea.se  of  the  bones  is  often 
found,  a  fact  which  is 
proven  bj'  the  X-ray  pict- 
ures. 

Since  secondary  tuber- 
culosis occurs  frequently 
in  cases  of  real  chronic 
articular  rheumatism,  it 
would  not  be  reasonable  to 
draw  comprehensive  con- 
clusions, either  from  the 
tuberculosis  of  the  internal 
organs,  or  from  a  positive 
tuberculin  reaction.  Great- 
er weight  should  be  attrib- 
uted to  a  positive  result 
from  intra-peritoneal  in- 
oculation of  a  guinea-pig 
with  the  synovial  fluid 
obtained  by  puncture,  al- 
though even  a  secondary 
tubercular  infection  might 
have  to  be  considered. 

How  far  the  presenta- 
tion  of  this  disease, 
"  Rhumatisme  tubercu- 
leux  "  by  the  French  (Ber- 
ard  and  Destot,  Barjon, 
Poncet,  Maillard)  is  consistent  with  the  facts,  only  further  investiga- 
tions will  show.  Furthermore  it  may  lie  mentioned  that  hrnmophiha 
in  connection  with  articular  iKrmorrhage  may  result  in  a  chronic 
arthropathy,  which  might  remind  one  of  chronic  rheumatism.  Ar- 
thritis urica  is  so  rare  in  children  that  we  may  forego  discussing 
it  here.  Whether  it  will  be  possible  to  eliminate  cUnically  from 
chronic  articular  rheumatism,  synovitis  chronica  villosa,  as  described 
by    Schiiller    (a    disease    which    is    said    to    originate    from    a    special 

11—32 


Primary  chronic  arthritis  (rhumatisme  nouveux*.  Boy 
four  years  old.  Nodular  swelliiiKs  of  the  finger-joints.  Swell- 
ing of  the  hand-,  knee-  and  ankle-joints.  Participation  of  the 
left    hip-joint.     Beginning  of  disease  m  the  third  year  (.Spitzy). 


498 


THE    DISEASES   OF   CHILDREN 


cone-shaped    bacillus)   will   have   to    be    determined    only    by   further 
investigations. 

Prophylaxis. — Etiologically  only  two  really  effective  factors  are 
known  to  us;  namely,  rheumatic  heredity  and  the  influence  of  damp  and 
cold  habitations.  The  essential  points  to  consider,  therefore,  against 
the  further  development  of  the  disease  in  children  afflicted  with  rheu- 
matism are  hygienic  precautions  in  the  dwelling  and  proper  clotliing, 
as  well  as  appropriately  guarding  the  child  against  inclement  weather. 
Fi(j.  115.  Treatment. — Only  cases  of 

the  first  group  are  amenable 
to  treatment  with  the  sahcy- 
lates,  as  aspirin,  etc.,  and  in 
cases  of  intercurrent  acute  at- 
tacks it  will  be  well  to  return 
to  these  remedies. 

If  the  saUcylates,  however, 
are  ineffective  in  an  attack 
of  acute  articular  rheumatism, 
at  its  very  outset,  and  the  dis- 
ease shows  a  tendency  to  as- 
sume a  chronic  course,  then  it 
is  advisable  to  place  the  joints 
into  a  position  of  repose,  and 
to  use  local  apphcations  of 
mesotan  (see  page  489). 

Later  it  is  well  to  start 
with  what  is  paramount  to  all 
other  treatment,  i.e.,  physical 
therapeutics;  these  procedures 
may  sometimes  be  efficiently 
assisted  by  the  quite  persistent 
administration  of  iodide  of 
sodium.  After  all,  the  most 
important  mode  of  treatment, 
and  one  which  must  be  adhered  to  with  great  patience,  is  gentle 
massage  and  careful  passive  motion.  At  the  same  time  a  local 
treatment  with  10  per  cent,  ichthyol  in  vaseline  may  be  useful.  This 
is  to  be  rubbed  in  at  night  and  covered  with  wadding,  next  morning 
shght  friction  with  French  brandy. 

Immobilizing  bandages  ought  to  be  avoided  as  far  as  possible 
except  in  cases  of  acute  exacerbations.  In  advanced  stages  of  the  dis- 
ease the  production  of  active  or  passive  hyperemia  proves  useful. 
Bier's  process  of  producing  congestion  (stasis),  or  preferably  hot  air  or 
electric  light  baths  may  be  considered. 


I'liniai;,  v\;r.,uir  arllint,*.  Girl  I'liP  a'l-l  a  lialf 
years  old,  Noiliiiar  swelling:  of  the  jtiinls  t'i  llic  hands 
and  feet.     Onset  in  the  right  elbow-joint. 


ACUTE  ARTICULAR  RHEUMATISM  499 

Very  favorable  but  of  course  only  temporary  results  have  been  ob- 
served from  the  use  of  daily  hot  sand  baths  at  30-35°  C.  (86-95°  F.); 
for  one  half  to  one  hour.  Occasional!}'  apphcations  of  fango  (fango 
poultices)  of  40°  C.  (104°  F.)  daily  around  the  joints  for  six  hours  have 
attained  some  success.  Frequent  very  warm  apphcations  of  such  poul- 
tices to  the  vertebral  column  occasionally  lead  to  excellent  results. 
As  alternatives  douches  deserve  consideration,  if  there  is  no  tendency 
toward  acute  relapses.  Some  French  authors  have  seen  results  ^vith  the 
galvanic  current.  In  conjunction  with  external  treatment,  an  internal 
or  subcutaneous  treatment  with  arsenic  may  be  tried.  Occasionally 
rectal  feeding  may  be  necessary  if  there  is  a  marked  involvement  of  the 
temporomaxillary  articulation. 

As  to  the  success  of  hydrotherapy  (as  Nauheim,  Tephtz,  Wildbad, 
etc.)  we  can  hardly  judge,  since  almost  without  exception  only  children 
of  the  poorer,  or  the  poorest,  classes  are  affhcted  with  the  disease.  If 
stiffness  of  the  joints  and  contractures  have  already  formed,  then 
very  beneficial  functional  results  can  be  obtained  by  orthopedic  and 
mechanical  treatment,  reduction,  tenotomy,  plastic  surgery  of  tendons, 
traction,  and  apparatus  (Spitzy,  Reiner). 

Possibly  thiosinamin  could  be  used  in  these  cases  to  great  advan- 
tage, although  so  far  as  I  know,  it  has  never  been  tried,  in  spite  of  its 
softening  influence  upon  the  cicatricial  and  connective  tissues,  especially 
as  a  transition  from  the  chronic  into  an  acute  inflammation  would  be 
welcomed. 

Menzer's  serum  has  not  as  yet  been  tried.  The  hypnotic  suggestive 
therapeutic  endeavors  of  some  authors  (Bernheim,  Grossmann)  can 
be  regarded  only  with  skepticism  especially  with  children. 

A  special  surgical  procedure,  i.e.,  injection  of  iodoform  guaiacol 
glycerin  emulsion,  or  a  free  opening  of  the  joints  and  excision  of  the 
villous  coat,  would  have  to  be  considered  in  a  case  of  Schiiller's  syno- 
vitis chronica  villosa. 

Von  Starck  saw  rapid  improvement  result  from  inunctions  of  ungt. 
Crede  in  a  case  presenting  the  picture  of  Still's  disease.  In  cases 
attended  with  fever,  the  use  of  colloidal  silver  in  the  form  of  Crede's 
ointment  or  intravenous  collargol  injections  would,  at  any  rate,  be 
worth  a  trial. 


SYPHILIS 

BY 

Dr.  C.  HOCHSINGER,  of  Vienna 

translated  by 
Dr.   JOSEPH   BRENNEMANN,  Chicago,  III 


The  chapter  on  Syphilis  of  Children  will  be  devoted  to  a  discussion 
of  all  those  changes  brought  about  by  sypliiUs  that  affect  the  human 
organism  from  the  time  of  conception  to  the  beginning  of  puberty. 

Sypliihs  in  childhood  may  have  its  origin  in  an  hereditary  trans- 
mission from  diseased  parents,  or  it  may  be  acquired  as  an  ordinary 
infection  through  contagion.  One  must,  therefore,  distinguish  between 
hereditary  and  acquired  syphiUs. 

HEREDITARY  SYPHILIS 

1.    THEORETICAL  CONSIDERATION  OF  THE   HEREDITARY 
TRANSMISSION  OF  SYPHILIS 

In  acquired  infantile  syphihs  there  is  a  single  mode  of  infection  just 
as  in  later  years,  i.e.,  contact  infection;  hereditary  sypliihs  on  the  other 
hand  may  be  transmitted  iij  two  ways.  We  may  have  a  germinal 
hereditary  transmission  through  the  germ-cells,  oi-  a  direct  intra- 
uterine infection.  Ever  since  Kassowitz's  epoch-making  work  on  this 
subject  (1876),  the  possibiUty  of  a  germinal  transmission  has  been  undis- 
puted, wliile  intra-uterine  infection  by  way  of  the  placenta  was  held  to 
play  a  subordinate  part.  Recently  Matzenauer  (1903),  as  Oedmansson 
did  formerly,  has  maintained  that  a  transmission  from  the  spermatozoa 
to  the  ovum  has  not  been  proven,  and  that  intra-uterine  infection  is  the 
only  conceivable  method  of  transmission  of  sypWhs  from  the  parent  to 
the  offspring.  He  supports  this  view  by  the  fact  that  a  purely  germinal 
transmission  is  unknown  in  any  other  infectious  disease.  His  main  thesis 
is:  "Without  maternal  syphihs,  there  is  no  hereditary  transmission 
of  the  disease  of  the  child." 

It  is  not  possible  in  a  work  of  this  kind  to  discuss  this  \'iew  in  de- 
tail: a  view  that  would  have  at  least  the  advantage  of  greater  simphc- 
ity  if  it  were  tenable.  I,  for  my  own  part,  must  hold  to  the  possibihty 
of  a  purely  paternal,  i.e.,  spermatic,  transmission  of  syphiUs,  a  conclu- 
sion that  is  based  on  many  years  of  careful  observation.     With  all  due 

500 


SYPHILIS  501 

respect  to  Matzenauer's  attempt  to  simplify  this  complex  question  of 
hereditary  transmission  of  syphilis  I  cannot  refrain  from  expressing  my 
conviction,  that  in  liis  zealous  endeavor  to  refer  all  questions  pertain- 
ing to  hereditary  syphihs  to  intra-uterine  infection  by  way  of  the  placenta, 
he  has,  in  more  than  one  way,  distorted  chnical  facts.  With  the  large 
and  constantly  increasing  literature  on  the  subject  of  hereditary  syph- 
ilis, it  is  impossible  to  go  into  details  and  mention  all  of  the  past  and 
present  authorities,  and  their  various  views.  It  will  be  possible  to 
take  only  a  general  survey  of  the  most  important  views  and  questions 
bearing  upon  the  subject.  As  to  terminology,  Solger  and  Martins 
maintain  that  if  Matzenauer's  view  were  accepted,  the  term  "hereditary 
syphilis"  would  be  incorrect,  and  "congenital  syphiUs"  should  be  put 
in  its  place,  since  only  such  disturbances  could  be  looked  upon  as  heredi- 
tary, as  had  been  transmitted  through  the  germ.  Schaudinn,  in  conjunc- 
tion with  Hoffman,  has  possibly  found  the  specific  cause  of  syphilis  in 
their  spirochcete  pallida.  The  demonstration  of  this  bacterium,  that  is 
characterized  by  a  special  form  with  narrow,  steep,  and  numerous  con- 
volutions (up  to  14),  is  most  satisfactorily  made  by  staining  with  a 
modified  Giemsa  stain  dried  specimens  obtained  from  the  tissue  juices 
of  eroded  syphilitic  primary  and  secondary  lesions.  Buschke  and  Fis- 
cher, Hoffmann,  Levaditi,  Salomon,  Leiner,  Nobecourt,  Ba3'et,  have  all 
found  the  characteristic  spirilla  in  the  contents  of  the  blebs  of  syphiUtic 
perapliigus.  M.  Oppenheim  and  0.  Sachs,  however,  could  not  find  them 
in  the  same  lesion.  In  the  Uver,  spleen,  lungs,  lymphatic  glands 
(Bertarelh  and  Volpino,  Bronnum,  Ellermann,  Reischauer,  Buschke,  W. 
Fischer),  and  in  the  blood  of  children  with  hereditary  syphiUs,  this 
parasite  has  been  seen,  so  that  Levaditi  considers  herechtary  syphilis 
as  a  spirillosis.  The  frequent  positive  findings  in  hereditary  syplailis, 
and  the  occurrence  of  spirochjete  pallida  in  the  inoculation  scleroses 
of  monkeys,  would  lead  one  to  attribute  to  tliis  parasite  a  more  impor- 
tant role  in  the  etiology  of  syphiUs,  than  to  the  other  nucroorganisms 
that  have  been  advanced  as  the  specific  cause  of  tliis  disease. 

Classification  of  Hereditary  Syphilis. — Two  factors  must  be  con- 
sidered in  the  hereditary  transmission  of  sypliilis: 

1.  The  hereditary  transmission  of  the  contagion,  which  leads  to 
genuine,  virulent  infection  in  the  offspring. 

2.  The  hereditary  transmission  of  certain  constitutional  changes 
that  have  been  brought  about  in  the  parent  by  the  specific  poison, 
these  changes  manifesting  themselves  in  the  offspring  as  more  or  less 
well  marked  general  disturbances  such  as  one  finds  in  the  offspring  of 
alcohohcs,  arthritics,  etc. 

Those  belonging  to  the  first  group  represent  congenital  syphihs  in 
the  narrower  sense.  This  may  be  divided  into  syphilis  that  has  mani- 
fested itself  during  intra-uterine  life,  and  that  which  has  appeared  only 


502  THE   DISEASES   OF   CHILDREN 

I 

post-partuni.  The  former  may  he  subdivided  into  syphilis  embryonalis, 
fcetalis,  and  neonatorum.  Tlic  latter,  according  to  the  views  of  many, 
should  be  subdivided  into  sypliilis  congenita  prsecox  and  tarda,  de- 
pending upon  whether  the  congenital  disease  first  manifested  itself 
shortly  after  birtii,  or  not  until  the  time  of  puberty.  That  the  latter 
form  has  in  no  way  been  proven,  may  be  stated  in  advance  at  this 
point.  There  is  still  less  evidence  of  an  inheritance  of  syphihs  by  the 
grandchild,  i.e.,  the  tliird  generation,  wliich,  if  it  did  exist,  would  form  a 
special  form  of  late  sypliihs. 

The  second  main  group  no  longer  depends  upon  changes  brought 
about  by  direct  hereditary  transmission  of  germs,  but  upon  the  devel- 
opment of  disease  and  of  dystrophic  conditions,  such  as  arrest  of  de- 
velopment, and  constitutional  disturbances,  which  do  not  themselves 
represent  syphihtic  affections,  but  are  connected  vnth,  and  dependent 
upon,  the  depraving  influence  of  sypliihs  upon  the  general  health  of  the 
parents  (A.  Fournier's  Parasypliihs).  Similar  symptoms  may  appear 
as  a  result  of  syphihtic  infection,  either  congenital  or  acquired,  later  in 
hfe,  so  that  besides  the  congenital  parasyphihtic  affections,  one  must 
distinguish  also  those  which  appear  later  in  hfe. 

Sources  of  Hereditary  Syphilis. — Hereditary  sypliihs  may  orig- 
nate  from  the  father,  or  from  the  mother,  or  from  both  at  the 
same  time. 

1.  Syphilis  from  the  Father. — Sypliilis  of  the  child  originating 
in  the  father  without  infecting  the  mother  (recently  denied  by  Matze- 
nauer),  depends  upon  spermatic  infection  of  the  ovule,  and  its  occur- 
rence is  demonstrated  by  the  fact  that  women  can  bear,  in  turn,  syphihtic 
and  healthy  children,  if  they  have  become  pregnant  first  by  a  man  with 
latent  sypliihs,  and  then  by  a  nonsyphihtic  man.  It  is  further  dem- 
onstrated by  the  striking  results  of  antisypliihtic  treatment  of  the  hus- 
band alone  in  famiUes  where  a  mother,  who  is  free  from  syphihs,  has 
given  birth  to  syphihtic  cliildren.  The  treatment  of  the  husband  alone, 
nearly  always  suffices  to  keep  the  later  offspring  free  from  syphihs. 

Although  we  are  not  famihar  at  the  present  time  vnt\\  the  real 
nature  of  spermatic  infection  of  the  ovum,  the  fact  that  women  who  are 
permanently  free  from  sypliihs  can  give  birth  to  sj'pliihtic  children,  is 
absolutely  undeniable  and  can  only  be  explained  by  the  hypothesis  of  a 
purely  paternal  transmission  of  syphihs. 

According  to  the  law  of  CoUes  and  Baumes  (1837  and  1840),  a 
mother  who  was  well  at  the  time  of  conception  acquires  immunity 
against  syphihs  by  being  pregnant  with  a  child  that  is  syphihtic  from 
its  father.  This  immunity  of  the  mother  is  frequently  looked  upon  as 
an  expression  of  infection  of  the  mother  through  conception,  and  the 
disease  itself,  under  these  circumstances,  is  spoken  of  as  conceptional 
syphihs  (A.  Fournier). 


PLATK  24. 


SYriiir.is  503 

According  to  A.  Matzenauor,  these  iniiiume  iiiotliers  have  become 
syphilitic  through  an  undiscovered  contact  infection  from  a  .s3plulitic 
husband,  and  for  tliis  reason  alone  arc  immune.  Even  if  it  is  entirely 
possible  that  the  primary  manifestations  should  be  overlooked,  it  would 
be  inconceivable  that  there  should  be  complete  and  lasting  absence  of 
all  syphilitic  symptoms  for  many  decades  in  women  that  have  remained 
untreated  and  have  been  observed  by  experienced  physicians.  I  con- 
sider such  mothers  simply  immune,  but  not  syphilitic.  (Observations 
in  4  of  my  own  families.) 

The  direct  paternal  transmission  of  syphilis  to  the  offspring,  de- 
pending upon  the  degree  of  virulence,  can  manifest  itself  in  death  of  the 
foetus,  or  in  evident  syphilitic  manifestations  at  birth,  or  after  birth, 
or  through  certain  parasyphilitic  symptoms  early  or  late  in  childhood. 

2.  Syphilis  from  the  Mother. — Several  possibilities,  according  to 
various  authors,  are  here  to  be  considered:  The  mother  may  have  been 
syphilitic  before  impregnation  (anteconceptional);  or  she  may  have  been 
infected  in  consequence  of  impregnation  (conceptional),  or  after  im- 
pregnation, i.e.,  during  pregnancy  (postconceptional). 

(a)  Anteconceptional  Syphilis. —  If  the  mother  is  syphilitic  and  the 
father  is  well,  one  might  think,  by  analogy  with  spermatic  syphilis,  of 
an  ovular  syphilis,  remembering  however  the  possibility  that  the  fcctal 
infection  may  have  l^een  transmitted  during  pregnancy  through  the 
placenta  of  the  diseased  mother  to  the  fcEtus.  The  view  formerly  ad- 
vanced by  Kassowitz,  that  the  placenta  constituted  a  barrier  between 
mother  and  child  through  wliich  the  contagion  of  sypliilis  could  not  pass, 
has  not  shown  itself  to  be  tenable.  When  the  placenta  itself  becomes 
diseased  there  is  no  longer  any  hindrance  to  fcctal  infection  along  the 
placental  route. 

(b)  Conceptional  Syphili.''. — This  term  is  used  by  many  authors  to 
designate  infection  of  a  woman  through  impregnation  by  a  sypliilitic 
man,  an  occurrence  that  is  wholly  unproven  and  incapable  of  proof. 

As  a  clinical  expression  of  conceptional  maternal  syphilis,  one  might 
think,  first  of  all,  of  the  occurrence  of  secondary  symptoms  without 
primary  lesion,  several  weeks  after  conception  (earl}'  conceptional 
sypliilis),  in  wliich  cases,  however,  one  could  not  exclude  an  unrecog- 
nized primary  lesion  following  ordinary  contact  infection. 

The  advocates  of  conceptional  maternal  sypliilis  accept  also  the 
possibility  of  a  late  form,  i.e.,  syphilis  appearing  many  years  after 
conception  in  the  mother  in  the  form  of  tertiary  manifestations  (Ter- 
tiarsme  d'Emblee,  A.  Fournier,  Finger,  von  Diiring,  and  others),  a  \iew 
even  less  demonstrable  than  that  of  an  early  conceptional  sypliilis. 

(c)  Postconceptional  Syphilis. — The  mother  is  infected  during  preg- 
nancy. The  foetus  may,  or  may  not,  become  sypliiUtic.  If  the  mother 
under  such  circumstances  transmits  her  disease  to  the  foetus  that  was 


504  THE   DISEASES   OF   CHILDREN 

primarily  health}-,  during  pregnancy,  tlien  we  have  a  real  intra-uterine 
infection.  If  the  mother  acquires  syphihs  during  the  early  periods  of  her 
pregnancy,  between  the  second  and  the  fifth  months,  then  the  chances 
are  greater  that  the  child  will  be  infected  within  the  uterus  than  if  the 
mother  acquires  the  disease  during  the  second  half  of  pregnancy.  Mater- 
nal infection  occurring  during  the  last  two  months  of  pregnancy  does  not 
seem  to  be  dangerous  to  the  child.  In  general,  one  must  remember  that 
intra-uterine  foetal  infection  is  by  no  means  a  necessary  sequel  to  the 
postconceptional  maternal  syphilis,  but  rather  a  facultative  one.  It 
is  certain  that  intra-uterine  transmission  of  syphilis  is  preceded  by  a 
specific  disease  of  the  placenta  which  causes  it  to  be  permeable  by  the 
contagion  of  sypliihs. 

In  a  case  observed  by  Oedman.sson,  congenital  syphihs  occurred  in 
the  child  after  infection  of  the  mother  at  the  beginning  of  the  third 
month  of  pregnancy. 

The  consequences  of  maternal  syphihs,  other  things  being  equal, 
are  considered  as  more  serious  to  the  offspring  than  those  of  paternal 
origin.  Intra-uterine  fcctal  death  and  severe  congenital  syphihs  are  said 
to  be  more  frequent  in  tlie  former  than  in  the  latter.  And  yet  recent 
maternal  sypliilis  acquired  during  pregnancy  is  very  frequently  without 
any  influence  upon  the  foetus,  so  that  a  healthy  child  may  be  born  in 
these  circumstances.  Such  observations  teach  that  the  placenta  is  in 
very  many  cases  a  protecting  filter  against  the  contagion  of  syphihs, 
and  only  when  is  becomes  diseased  can  intra-uterine  foetal  infection 
take  place. 

3.  Syphilis  mixta. — Father  and  mother  are  both  syphilitic  before 
conception.  The  severity  and  certainty  of  infection  of  the  child  is  here 
in  proportion  to  the  recentness  of  parental  infection.  The  germinal 
impregnation  method  of  infection  unites  with  that  of  intra-uterine  infec- 
tion in  a  combined  action  on  the  foetus.  In  this  method  of  infection 
there  can  hkewise  occur  in  the  cliild,  on  theoretical  grounds,  genuine 
virulent  manifestations  of  syphihs  and  parasypliihtic  dystrophies. 

Immunity  to  Syphilis  of  Mother  and  Foetus. — One  sees  very  fre- 
quently children  who  were  born  to  mothers  that  had  recently  become 
syphihtic,  that  are  free  from  all  evidence  of  syphilis  and  remain  so,  and 
on  the  other  hand  mothers  who  are  healthy  and  remain  free  from  syphihs 
and  yet  give  birth  to  cliildren  that  are  severely  syphihtic.  In  the  latter 
case  we  have  to  do  with  a  fcetus  infected  spermatically,  while  the  mother 
escaped  from  a  contact  infection  with  the  specific  factor.  In  this  man- 
ner the  mother  acquires  a  liigh  degree  of  immunity  against  sypliihs, 
so  that  she  can  usually  nurse  her  own  specifically  infected  baby  with 
impunity,  while  a  wet-nurse  would  invariably  become  infected  by  such 
a  child,  in  accordance  with  the  law  of  Colles  and  Baumes.  Exceptions 
to  this  law,  usually  in  primiparse,  doubtless  do  occur,  in  spite  of  the 


SYPHILIS  505 

protest  of  Matzenauer,  and  these  can  then  serve  as  the  crowning  evidence 
in  favor  of  the  possibilit}'  of  a  purely  paternal  transmission  of  syphilis. 

It  remains  to  be  decided  whence  this  maternal  immunity  arises. 
A  number  of  authors  hold  the  view  based  on  Colles'  law  that  these 
mothers  are  syphilitic  and  consider  the  disease  as  either  latent  and  due 
to  contact,  or  as  conccptional.  Others  again  would  by  no  means  iden- 
tify this  immunity  with  latent  syphihs  and  would  explain  this  immunity 
of  Colles'  by  assuming  the  transmission  of  immunizing  substances  (an- 
titoxins) from  a  paternally  syphilitic  foetus  to  the  mother  during  preg- 
nancy. These  mothers  would  therefore  be  immune  to  syphilis  without, 
however,  being  syphilitic. 

Whether  this  immunity  in  mothers  who  remain  free  from  the  dis- 
ease and  yet  give  birth  to  congenitally  syphihtic  children  is  permanent, 
or  temporary,  remains  undecided.  Probably  it  is  only  transitory,  but 
nearly  always  extends  beyond  the  period  of  nursing.  Even  if  the  mother 
does  not  become  infected  later  in  life  in  spite  of  continued  cohabitation 
with  a  sypliilitic  husband  this  by  no  means  is  proof  of  a  permanent  im- 
munity. If  after  the  period  of  nursing  the  child  is  properly  treated  and 
later  in  life  is  kept  free  from  virulent  manifestations,  then  there  is  no 
longer  any  opportunity  for  infection  of  the  mother  from  the  cluld.  The 
husband,  however,  in  such  cases  is  usually  long  before  tliis  free  from 
infectious  products,  and  it  would  be  making  a  false  deduction  to  main- 
tain that  all  mothers  of  paternally  syphihtic  children  are  immune 
throughout  hfe  simply  because  they  remain  free  from  sypliihs.  In  tliis 
view  is  found  an  answer  to  that  objection  to  the  existence  of  a  pure 
Colles  immunity  which  states  that  the  action  of  antitoxins  could  give 
only  a  transient  protection  such  as  would  follow  vaccination. 

Profeta's  law  attributes  to  the  healthy  cliild  of  a  recently  syphi- 
htic mother  immunity  to  syphihs  and  maintains  that  this  immunity 
may  even  extend  to  all  of  the  offspring  of  sypliihtic  parents.  This  view 
is  not  tenable  since,  as  Matzenauer  has  rightly  stated,  a  germinal  trans- 
mission of  immunity  is  unthinkable — cluldren,  born  of  syphihtic  fathers, 
but  of  healthy  mothers,  that  are  healthy  and  not  immune,  cannot  for 
tliis  reason  in  any  way  be  considered  as  exceptions  to  Profeta's  law. 
This  law  has  notliing  approximating  the  authority  of  the  law  of  Baum6s 
and  Colles,  and  all  the  less  so  since  undoubted  cases  of  sypliihtic  rein- 
fection of  congenitally  sypliilitic  individuals  are  known  (Hochsinger, 
E.  Lang,  von  During,  Tschlenow,  etc.). 

Since,  in  these  children  remaining  free  from  syphilis  yet  born  to 
syphihtic  mothers,  we  have  a  transmission  of  soluble  immunizing  sub- 
stances from  the  diseased  mother  to  the  healthy  foetus  by  wa.y  of  the 
placenta,  just  as  in  the  case  of  healthy  mothers  of  paternally  sypliihtic 
children,  it  is  impossible  to  assume  a  hfelong  immunity.  In  both  cases 
the  degree  of  protection  depends  upon  the  duration  and  the  amount  of 


506  THE   DISEASES   OF   CHILDREN 

the  action  of  the  antitoxin  under  consideration.  Regarded  from  this 
standpoint  the  exceptions  to  CoUes'  law  as  well  as  to  Profeta's  law,  are 
in  no  way  surprising,  indeed  from  a  theoretical  standpoint  such  excep- 
tions are  to  be  expected. 

Hereditary  Transmissibility. — In  general,  the  abihty  to  trans- 
mit sypliilis  to  the  offspring  is  proportional  to  the  abihty  to,  ])roduce  con- 
tact infection,  i.e.,  contagion.  It  is  essentially  associated  with  the  sec- 
ondary stage,  but  by  no  means  does  it  always  follow,  and  in  the  tertiary 
stage  only  rarely  so. 

The  general  rule  laid  down  by  Kassowitz  that  the  degree  of  trans- 
missibiUty  of  sypliihs  gradually  diminishes  in  proportion  to  the  duration 
of  the  chsease,  remains,  on  the  whole,  correct,  even  if  there  are  excep- 
tions. In  sypliilitic  famihes  one  sees  as  a  rule  first  abortions,  then 
stillbirths,  then  hving  premature  infants,  then  living  syphihtic  infants, 
then  Uving  infants  free  from  syphihs  or  not  manifesting  symptoms  till 
after  birth,  and  finally  children  that  remain  free  from  syphilis.  To  this 
rule  one  finds  many  exceptions,  as  the  birth  of  healthy  children  in  the 
midst  of  those  that  are  syphihtic.  Tliis  is  spoken  of  as  alternating  trans- 
mission, and  is  considered  by  Matzenauer  as  one  of  the  proofs  of  a  purely 
maternal  transmission. 

The  severity  of  the  disease  in  the  cliild  depends  upon  the  nature  and 
manner  of  acquiring  it  and  the  time  at  wliich  it  occurs  in  the  before- 
mentioned  scale.  Cliildren  that  are  only  sUghtly  diseased  often  are  born 
apparently  well  and  do  not  give  evidence  of  sypliilis  until  some  time  dur- 
ing the  first  three  months.  When  both  parents  are  sypliihtic,  we  have 
the  conditions  that  most  frequently  lead  to  manifestations  in  the  cliild, 
according  to  Fournier  in  92  per  cent,  of  cases.  In  purely  maternal  syph- 
ihs this  occurs  in  84  per  cent,  of  cases  according  to  Fournier,  and  in 
purely  paternal  sypliihs,  in  37  per  cent,  of  cases. 

In  72  marriages  of  fathers  who  were  sypliihtic  and  mothers  who 
remained  free  from  the  disease,  in  the  series  of  cases  that  I  have  observed, 
there  were  110  stillbirths  and  197  hving  infants.  In  65  per  cent,  of  the 
marriages  in  which  the  father  alone  was  syphihtic  there  were  stillbirths; 
in  35  per  cent,  there  were  Uving  cliildren  only.  In  my  series  of  26  fami- 
hes in  which  there  was  positive  maternal  syphihs  19  mothers  gave  birth 
to  34  dead  babies.  According  to  my  experience  there  is  no  essential 
difference  as  far  as  death  of  the  fcetus  is  concerned  between  purely  pater- 
nal and  purely  maternal  transmission  of  syphihs.  In  67  famihes,  that 
I  have  observed,  in  wliich  the  parents  were  S3'pliihtic  there  were  266 
pregnancies;  142  children  were  born  ahve;  76  died  during  the  first  few 
days;  and  there  were  48  abortions,  making  a  total  of  124  stillbirths 
in  266  pregnancies. 

It  is  generally  accepted  that  maternal  syphihs  loses  its  effect  upon 
posterity  less  rapidly  than  that  in  which  the  father  alone  is  affected,  so 


SYPHIIJS  507 

that  a  syphilitic  woman  who  marries  a  second  time  and  becomes  preg- 
nant by  a  healthy  man,  still  frequently  gives  birth  to  infected  children, 
and  thus  really  transmits  the  disease  from  her  first  husband  to  the  ofT- 
spring  to  the  second.  Some  authors  claim  to  liave  seen  transmission  of 
syphihs  in  a  virulent  form  more  than  twenty  years  after  the  mother  was 
first  infected. 

With  reference  to  the  influence  upon  posterity  of  congenital  syphi- 
liti,cs,  one  might  tliink,  from  a  theoretical  standpoint  according  to  Fin- 
ger, of  the  transmission  of  genuine  virulent  manifestations  of  sypliilis; 
of  the  production  of  paras3'philitic  symptoms;  and  finally,  of  the  oc- 
currence of  a  congenital  immunity  to  syphihs.  The  possibihty  of  trans- 
mission to  the  third  generation  is  wholly  without  proof.  Its  occurrence 
could  be  accepted  as  demonstrated  only  when  a  mother  who  is  known 
to  be  congenitally  sypliihtic  gives  birth  to  a  syphihtic  child,  while  the 
father  of  the  child  is  known  to  be  free  from  syphihs,  and  the  mother 
has  not  been  specifically  reinfected.  Still  less  evidence  is  there  in  favor 
of  the  view  frequently  expressed  that  sypliihs  of  the  grandparents  can 
produce  dystrophy  and  immunity  to  the  disease  in  the  grandchildren, 
i.e.,  in  the  tliird  generation.  In  the  whole  consideration  of  whether 
syphihs  can  be  transmitted  to  the  tliird  generation  either  in  the  form  of 
genuine  \irulent  sypliihs,  or  as  parasj'phihtic  manifestations,  too  httle 
attention  has  been  paid  to  the  state  of  health  of  the  second  generation. 
Hereditary  sypliihs,  in  the  first  place,  must  be  demonstrated  in  the 
second  generation  so  as  to  leave  no  doubt;  acquired  syphilis,  on  the 
other  hand,  must  be  excluded  with  equal  certainty,  both  as  to  infection 
in  an  inthvidual  previously  well,  and  as  to  reinfection  in  one  already 
congenitally  syphihtic.  The  same  naturally  ajjphes  equally  to  the  third 
generation. 

2.     FCETAL   SYPHILIS 

In  this  chapter  will  be  discused  those  changes  brought  about  by 
the  action  of  the  transmitted  syphihtic  poison  upon  the  fa>tal  organism, 
from  the  time  of  the  formation  of  the  ovum  to  the  time  of  birth.  There 
is  here  always  the  expression  of  severe  infection  of  the  foetus  caused  by 
recent  sypliilis  in  the  parents.  The  gravit}^  of  syphihtic  manifestations 
in  the  foetus  is  due  to  specific  changes  in  the  A-iscera,  which  changes  are 
usually  absent,  or  only  shghtly  present,  in  those  cases  beginning  after 
birth.  In  fcetal  sypliihs  there  is  a  striking  affinity  of  the  infectious 
material  for  the  large  glandular  organs  and  for  the  gro\\'ing  portions  of 
the  osseous  system,  while  tlie  skin,  which  is  a  favorite  place  for  an  attack 
after  birtli  is  relatively  immune  before  birth.  There  is  developmental 
ground  for  this  in  that  these  organs  wliich,  at  the  time  of  the  formation 
of  the  specific  poison  in  the  organism,  show  a  pecuhar  liyperaMiiia, 
either  functional,  or  associated  with  growth,  take  up  the  poison  with 
especial  avidity.     If  the  contagion  manifests  itself  in  an  early  jieriod 


508  THE   DISEASES   OF   CHILDREN 

of  foetal  life,  then  those  internal  glandular  organs,  the  lungs,  liver, 
kidneys  and  pancreas,  that  develop  early  are  involved.  Later,  on  ac- 
count of  the  rapid  growth  in  length  of  the  foetus,  there  appear  changes 
at  the  epiphyseal  borders  in  the  liollow  bones.  The  skin,  on  the  other 
hand  does  not  really  develop  its  glandular  apparatus  till  the  later 
months  of  intra-uterine  Ufe,  when  it  is  preparing  for  its  extra-uterine 
life,  and  so  does  not  show  characteristic  changes  till  shortly  before  or 
after  birth. 

General  Characteristics  of  Early  Congenital  Syphilis. — If  one 
bears  in  mind  the  embryological  conditions,  it  is  a  simple  matter 
to  find  a  satisfactory  explanation  of  the  genesis  of  the  early  lesions  of 
hereditary  syphihs.  As  opposed  to  acquired  sypliihs,  the  typical  lesion 
of  early  hereditary  syphihs  is  found  in  a  diffuse  cell  proHferation  having 
its  origin  in  the  perivascular  connective  tissue  of  the  smallest  vessels, 
i.e.,  the  mesenchyma.  For  this  reason  one  very  rarely  sees  a  soUtary 
syphiloma  in  t-he  foetus,  or  in  the  young  infant,  but  rather,  almost  in- 
variably, diffuse  cell  proHferation  and  inflammation. 

It  is  a  mistake  to  consider  the  visceral  and  bone  changes  of  foetuses 
and  of  newborn  and  young  infants  as  tertiary,  and  the  skin  manifes- 
tations as  secondary  lesions,  because  they  are  identical  with  those 
occurring  in  these  structures  in  acquired  syphihs.  The  diffuse  charac- 
ter of  those  lesions  of  early  hereditary  syphihs,  no  matter  where  local- 
ized speaks  for  a  single  uniform  genesis,  excluding  the  possibihty  of  a 
division  into  secondary  and  tertiary  lesions.  The  predilection  of  this 
inherited  contagion  as  determined  by  embryological  conditions,  for  those 
tissues  that  are  especially  characterized  by  marked  vascularity  and  rapid 
growth  during  tliis  period,  speaks  for  the  assumption  that  in  the  le- 
sions of  early  congenital  sypliihs  we  have  to  do  with  a  single,  uniformly 
irritating  action  of  the  specific  poisonous  substance,  wliich  is  earhest 
and  most  active  wherever  there  is  the  greatest  afflux  of  tissue  juices. 
Tliis  has  notliing  in  common  with  the  usual  classification  of  syphihs 
into  stages. 

The  anatomical  picture  of  the  changes  occurring  in  early  congenital 
syphihs  is  an  identical  one  in  all  organs.  The  most  essential  changes 
are  found  in  the  hver,  lungs,  kidneys,  pancreas,  spleen,  thymus,  and  at 
the  growing  points  in  the  bony  system.  Two  kinds  of  lesions  are  most 
prominent: 

1.  Diffuse  cell  prohferation,  starting  from  the  smallest  blood  ves- 
sels, in  the  interstitial  connective  tissue  of  these  organs  with  a  decided 
tendency  to  later  contraction  and  to  prominent  participation  on  the  part 
of  the  vascular  system.  In  the  small  blood  vessels  this  prohferating 
process  begins  in  the  outer  walls  in  the  form  of  a  cuff  and  regularly  ad- 
vances peripherally  toward  the  connective  tissue,  more  rarely  toward 
the  inner  wall  of  the  vessels,  frequently  leading  to  obhteration  (Fig. 


SYPHILIS 


509 


116).  In  the  bone  changes  and  in  those  of  the  skin  we  have  an  iden- 
tical process,  as  will  be  shown  later,  although  the  conditions  are  not  so 
evident  at  a  glance  as  they  are  in  the  case  of  the  viscera. 

In  all  affected  organs  one  may  have  locaUzed  denser  collections  of 
cells  wliich  are  recognizable  even  macroscopically  and  are  often  spoken 
of  as  iniUary  sypliilomata,  but  are  not  gummata. 

This  diffuse  cell  proUferation,  or  hypertrophy,  of  the  mesenchyma 
which  can  so  pervade  whole  organs  of  stillborn  syplrihtic  infants  that  the 


Fig.  116. 


Lung  of  a  syphilitic  infant  of  the  ninth  month.  White  pneumonia,  (a)  A  bronchus  surrounded  by 
diffusely  infiltrated  lung  tissue  and  devoid  of  mucous  membrane.  The  epithelium  is  in  direct  contact  with 
the  hyperplastic  connective  tissue.  C6'  6)  Separateti  cylindrical  epithelial  cell  fibres.  Remains  of  foetal  epithelial 
tubules,  (c)  Larger  blood  vessels  with  diseased  walls  in  the  supporting  tissue  of  the  lungs,  id)  Small  arteries 
in  infiltrated  connective  tissue,  (e'  e)  Alveolar  spaces  packed  with  desquamated  epithelium  undergoing  fatty 
degeneration  and  in  part  disintegrated,  in  part  united  into  flattened  masses. 


parenchyma  is  no  longer  recognizable,  was  interpreted  by  Karvonen  as  a 
foetal  arrest  of  development  of  the  mesenchmya  and  not  as  an  inflamma- 
tory process  involving  the  supporting  tissue  of  the  developing  paren- 
chyma, as  Hecker  and  I  teach.  The  same  author,  later  Hecker,  Terrier 
and  Erdmann,  pointed  out  the  physiological  richness  in  round  cells  of 
the  foetal  parenchyma.  Since,  however,  these  organs  in  sypliiUtic  fa>- 
tuses,  in  which  the  cell  infiltration  of  the  interstitial  connective  tissue 
is  often  a  very  extensive  one,  are  heavier  and  larger  than  those  that 
are  not  sypliiUtic,  one  cannot  doubt  that  the  pathological  nature  of  tliis 
hyperplasia  is  that  of  an  inflammatory  proUferation. 


510  THE   DISEASES   OF   CHILDREN 

2.  In  the  fcctal  organs  involved  in  this  hj'perplastic  process  there 
are  characteristic  and  pecuUar  arrests  of  development  of  the  paren- 
chyma. Incomplete  development  of  the  Malpighian  bodies,  persistent 
epithelial  ducts  and  the  formation  of  cysts  in  the  renal  cortex,  masses  of 
epithehal  cells  that  have  been  separated  off  and  isolated,  in  the  lungs, 
hver,  kidneys,  pancreas  and  gastro-intestinal  tract  and  cyst  formations 
lined  with  epithehum  in  the  thymus  may  all  be  mentioned  here.  It 
is  certain  that  the  hyperplasia  of  the  connective  tissue  areas  goes  hand 
in  hand  with  a  hypoplasia  of  the  parenchyma.  The  growing  osseous 
system  of  the  fa?tus  and  of  the  young  infant  shows  similar  disturbances 
of  development. 

Hereditary  syphihtic  changes  of  the  visceral  organs  of  fcetuses 
frequently  are  not  demonstrable  macroscopically.  Only  when  we  have 
circumscribed,  focal  collections  of  cell  infiltration,  and  the  formation 
of  hard  elevations,  like  callosities,  is  the  diagnosis  easy.  At  other  times 
there  is  simply  an  increase  of  volume  and  consistency,  most  constantly 
in  the  hver  and  spleen,  the  weight  of  wliich  as  compared  with  the  body 
weight  is  greater  than  normal  in  congenitally  sypliiUtic  foetuses.  The 
ratio  of  the  weight  of  the  hver  to  the  weight  of  the  fcctus  is  normally 
as  1 :  21 .5,  in  sypliihs  as  1 :  14.7 ;  that  of  the  spleen  is  normally  as  1 :  325, 
in  syplaihs  neonatorum  as  1 :  198. 

The  liver  of  syphihtic  foetuses  is  always  permeated  by  a  large 
amount  of  interstitial  cell  infiltration,  the  dependence  of  which  upon 
the  vascular  system  is  here  very  evident.  One  frequently  finds  in  this 
organ  wliich  is  usually  very  vascular,  small  yellowish  masses  from  the 
size  of  a  hemp-seed  to  that  of  the  head  of  a  pin,  composed  of  cloudy 
and  necrotic  hver  cells  surrounded  by  inflammatory  cells  arranged  about 
them  as  a  focus.  These  are  pecuUar  exudative  formations  that  occur 
solely  in  early  hereditary  syphihs,  and  are  to  be  interpreted  as  areas  of 
anaemic  necrosis.  Very  similar  areas  of  necrosis  are  found  in  the  kid- 
neys, especially  however  in  the  suprarenal  bodies,  and  also  in  the  epiphy- 
seal cartilages  and  in  the  cartilaginous  ends  of  the  bones  of  syphihtic 
dead  liorn  children. 

More  rarely  there  occur  well  developed  sclerotic  processes,  i.e., 
contractions  in  sypliihs  of  the  fa'tal  hver.  An  indurative  enlargement 
of  the  spleen  and  pancreas  is  frequent.  In  the  Icidneys,  besides  the  con- 
stant part  taken  by  the  vascular  system  in  the  form  of  a  diffuse  peri- 
vascular infiltration,  there  is  practically  always  present  an  incomplete 
development  of  the  cortical  parenchyma  with  rudimentary  develop- 
ment of  the  Malpighian  bodies  and  of  the  tubular  system. 

The  lung  frequently  shows  characteristic  changes  that  make  it  re- 
semble sarcomatous  tissue,  due  to  the  uniform  infiltration  with  round 
lymphoid  cells  (Ziegler).  Enclosed  within  these  areas  of  interstitial 
cell  infiltration  are  found  remnants  of  foetal  lung  tissue  from  a  former 


SYPHILIS  511 

period  of  development,  in  the  form  of  masses  of  cylindrical  or  cubical 
epitheliomata,  or  epithelial  tubules.  Another  change  results  from  a  com- 
bination of  an  extensive  desquamation  of  the  alveolar  epithehum  wlrich 
has  undergone  fatty  granular  degeneration  and  cell  proliferation  in  the 
interalveolar  lung  tissue,  from  which  there  results  a  uniform  whitish  gray 
discoloration  of  the  affected  portion  of  the  lung  and  the  pecuhar  homog- 
eneous appearance  of  the  cut  surface  (pneumonia  alba,  see  Fig.  116). 
Such  lungs  may  even  have  undergone  respiratory  movements,  and  are 
occasionally  found  in  congenitally  sypliihtic  infants  that  have  lived  for 
a  number  of  days.  One  must  not  forget  however  that  other  kinds 
of  pneumonia  may  occur  in  newborn  syphilitic  infants. 

Cyst-Hke  structures  in  the  thymus  are  very  characteristic  of  heredi- 
tary syphihs.  They  are  filled  with  a  secretion  that  resembles  pus  and 
are  to  be  interpreted  as  epithehal  spaces  of  the  foetal  thymus  separated, 
or  pinched  off,  by  inflammatory  cell  proliferation. 

Similar  perivascular  hyperplasias  and  parenchymatous  hypoplasias 
occur  hkewise  in  the  central  nervous  system,  in  the  gastro-intestinal 
mucous  membrane,  and  in  the  testicles  and  epididymis.  The  lesions 
of  the  osseous  system  will  be  discussed  in  a  connected  manner  in  a 
later  chapter. 

Death  of  the  Foetus  due  to  Syphilis.  -Tliis  can  occur  at  any 
period  of  intra-uterine  life,  but  is  most  frequent  between  the  fourth  and 
the  seventh  months  of  pregnancy.  A.  Fournier  found  230  abortions 
among  527  sypliilitic  pregnancies;  Le  Pileur  154  abortions  or  still- 
births among  414  syphilitic  pregnancies;  and  Coffin  27  dead  premature 
infants  out  of  28  pregnancies.  Habitual  abortion  is  to  be  attributed  to 
sypliilis  in  the  great  majority  of  cases. 

In  such  infants  born  dead  during  the  first  half  of  pregnancy  anatom- 
ical changes  in  the  foetus  are  not  always  clearly  marked  and  are  often 
demonstrable  only  when  histological  sections  are  compared  with  those 
from  syphilitic  foetuses  of  the  same  age.  These  changes,  however,  are 
never  absent  during  the  second  half  of  pregnancy.  Severe  general  in- 
toxication is  responsible  for  death  in  the  first  case,  but  especially  so 
is  an  early  involvement  of  the  placenta.  Both  maternal  and  fo-tal  por- 
tions of  the  latter  can  become  cUseased,  and  especially  so  in  cases  of  a 
purely  spermatic  infection  and  of  one  that  had  an  intra-uterine  origin. 
The  syphilitic  fa?tus  digs  its  own  grave  in  its  mother's  womb  by  means  of 
early  involvement  of  the  placenta,  by  changes  in  its  blood  vessels,  by 
prohferating  granulations,  by  the  formation  of  callosities  and  finally  by 
contractions,  that  impede  circulation.  Apart  from  these  specific  changes 
which  will  be  described  later,  all  kinds  of  developmental  disturbances  can 
occur  in  these  sypliihtic  premature  and  stillborn  fret  uses,  such  as  spina 
bifida,  anencephalus,  harelip,  clubfoot,  congenital  heart  disease,  and 
monstrosities  of  all  kinds. 


512  THE    DISEASES   OF   CHILDREN 

Frequently  the  cause  of  these  early  premature  and  still  births  is 
found  in  hydranmios  resulting  from  an  early  phlebitis  of  the  unibiUcal 
vein,  which  in  turn  is  dependent  upon  specific  changes  in  the  placenta. 

Changes  in  the  placenta  are  regularly  found  in  foetal  syphiUs.  The 
placenta  is  larger  and  heavier  than  noi'inal.  It  is  pale,  has  deformed 
lobes,  is  frequently  yellowish  in  color,  and  the  umbihcal  cord  is  hard  and 
thickened.  Histologically,  the  placental  blood  vessels  show  perivascular 
cell  infiltration,  and  a  pathological  condition  of  the  intima;  the  pla- 
cental parenchyma  shows,  further,  diffuse  or  nodular  masses  of  cells 
antl  extensive  foci  of  tissues  that  have  undergone  fatty  degeneration. 
In  the  umbilical  cord  are  frequently  found  perivascular  infiltrations  and 
characteristic  changes  in  the  blood  vessels,  on  which  alone  the  diagnosis 
of  hereditary  syphihs  can  be  made  if  there  is  doubt  otherwise  as  to  the 
cause  of  foetal  death. 

The  Hving  offspring  of  syphilitic  parents,  though  they  may  show  no 
chnical  evidence  of  the  disease,  very  frecjucntly  manifest  constitutional 
inferiority  as  shown  by  general  physical  weakness.  Among  48  syphi- 
litic children  born  ahve  in  Tarnier's  clinic  in  1900  and  1901  fourteen 
had  a  normal  weight  (not  under  3250  grams),  38  an  abnormally  small 
weight,  15  of  these  weighing  less  tlian  2500  grams.  The  losses  in  weight 
of  these  foetuses  is  the  more  striking,  because  they  have  regularly  severe 
visceral  affections  wliich  lead  to  an  increase  in  weight  of  the  larger 
glands  (Hecker,  Ho6hsinger). 

In  a  few  premature  or  full  term  infants  characteristic  changes  are 
found  in  the  skin  and  mucous  membranes  at  birth.  The  most  impor- 
tant skin  lesion  in  this  connection  is  sypliiUtic  pemphigus.  More  rarely 
these  children  are  born  with  a  papular  eruption.  The  most  prominent 
congenital  lesion  of  the  mucous  menibranes  is  syphihtic  coryza.  Very 
frequently  affections  of  the  bones,  of  the  eyes,  and  of  the  nervous  sys- 
tem are  present  at  birth,  to  say  nothing  of  those  that  involve  the  liver, 
the  spleen,  the  pancreas  and  the  intestinal  nmcous  membranes. 

3.     SYPHILIS  IN   INFANCY 

Two  kinds  of  organic  changes  are  to  be  considered : 

(a)  Those  that  are  carried  over  from  the  foetal  to  the  extra-uterine 
period,  especially  involvement  of  the  viscera,  of  the  osseous  system  and 
of  the  nose. 

(b)  Those  that  appear  after  a  period  of  latency  in  infants  appar- 
ently free  from  syphihs  at  birth,  especially  lesions  of  the  skin  and  mu- 
cous membranes. 

The  period  of  eruption  in  hereditary  syphilis  deserves  a  brief  general 
discussion  in  cases  of  the  second  group.  There  are  cliildren  that  are  l)orn 
free  from  sypliihs  from  a  chnical  standpoint,  that  develop  after  several 
weeks  or  months  an  eruption  similar  to  that  occurring  in  acquired  syphilis. 


PLATE  25. 


5.  a 


SYPHILIS  513 

The  first  appearance  of  tliis  eruption  is  ahvays  during  the  first  three 
months  of  life.  Most  frequently  it  starts  between  the  second  and  sixth 
week  after  birth. 

The  first  eruption  is  not  always  the  first  manifestation  of  the  dis- 
ease which  may  have  appeared  earher  in  the  form  of  specific  lesions  of 
the  nose,  viscera,  or  bones.  In  fact,  the  nose  is  nearly  always  invt)lved 
before  the  skin  eruptions  appear.  It  must  be  remembered  too  that 
syphihs  can  run  its  course  in  infancy  without  any  .skin  eruption  whatever. 

The  most  prominent  symptom  of  infantile  syphiUs  is  found  in  a 
rhinitis  that  consists  of  an  inflammation  of  the  na.sal  mucous  membrane, 
accompanied  by  hypertroph3\  Tliis  very  frequently  begins  during  intra- 
uterine hfe  and  is  accompanied  by  disturbances  of  development  of  the 
skeleton  of  the  nose. 

My  own  material  bearing  upon  this  point  comprises  256  cases  of 
hereditary  syphihs.  I  can  recall  no  case  in  which  this  hyperplastic 
rhinitis  was  absent.  Of  173  cases  of  specific  coryza  that  are  accuratclj* 
described  in  my  records,  65  can  be  used  in  determining  the  time  at  which 
this  symptom  first  appeared. 

In  38  cases  the  coryza  was  present  at,  or  very  shortlj'  after  birth. 

In  5  cases  it  appeared  one  week,  in  4  cases  two  weeks,  in  4  cases 
three  weeks,  and  in  2  cases  four  weeks,  after  birth.  In  53  cases  then  it 
appeared  during  the  first  month,  in  the  remaining  12  it  occurred  during 
the  fifth,  sixth  and  seventh  weeks. 

The  affection  begins  with  swelhng  of  the  nasal  mucous  membrane 
especially  of  the  inferior  turbinate  bone.  At  first  there  is  no  secre- 
tion, but  later  there  occurs  a  tough  sanguinopurulent  discharge  with 
a  tendency  to  the  formation  of  crusts.  There  is  a  very  characteristic 
snuffling  sound  later  accompanied  by  a  moist  ratthng  sound  due  to 
mucus.  This  not  infrequently  permits  the  diagnosis  of  hereditary 
sypMUs  at  a  distance.  This  impeded  nasal  respiration  makes  nursing 
difficult,  and  the  cliild  frequently  turns  the  head  back  and  holds  it  in 
a  position  of  opisthotonos  in  order  to  faciUtate  respiration. 

Tins  rliinitis  may  go  no  further  than  the  stage  of  swelling,  without 
any  pus  formation,  or  it  may  lead  to  ulceration  and  even  to  involvement 
of  the  cartilaginous  and  bony  skeleton  of  the  nose  with  resulting  changes 
of  shape  of  the  external  nose  (see  figures  121,  122,  132  and  133). 

As  a  result  of  cicatricial  contraction  of  the  cartilaginous  and  soft 
portions  we  have,  first  of  all,  the  pug  nose.  If  the  cartilaginous  septum 
contracts  completely  a  permanent  deformity  of  the  nose  may  result,  so 
that  the  softer  portion  may  form  only  a  short  projection  beyond  the  bony 
portion  with  the  nostrils  directed  upwards  (bucknose).  If  the  bony 
septum  is  made  smaller  through  rarification  and  ulceration,  or  through 
imperfect  development,  there  results  the  deformity  spoken  of  as  saddle 
nose,  characterized  by  a  depression  of  the  ridge  of  the  nose.     Perfora- 

11—33 


514 


THE   DISEASES   OF   CHILDREN 


tions  of  both  cartilaginous  and  bony  portions  of  the  septum  occur  in 
early  hereditary  sypliilis.  A  certain  number  of  these  cliildren  are  born 
with  deformities  of  the  nose,  frequently  with  abnormally  small,  or  ab- 
normall}^  flat  noses.  That  which  characterizes  these  noses  is  the  fact 
that  the  ridge  seems  pecuUarly  broad  and  deeply  sunken  between  the 
orbits  and  that  the  two  nasal  passages  meet  under  the  ridge  of  the  nose 
at  a  very  obtuse  angle.  The  cause  of  this  congenital  nasal  deformity  lies 
in  an  imperfect  foetal  development  of  the  cartilaginous  portion  of  the 
septum,  analogous  to  the  conditions  in  myxoedema  and  mongolian  idioc}-. 
The  skin  lesions  in  hereditary  syphihs  are  very  characteristic. 
Certain  forms  of  these  appear  only  in  the  congenital,  never  in  the 
acquired    disease.      These    are    syphilitic    pemphigus    and    a    diffuse 

infiltration  of  the  skin. 

One  nmst  distinguish 
in  early  hereditary  syphihs 
of  infants  between  diffuse 
and  circumscribed  skin 
lesions.  Wliile  the  latter, 
on  the  whole,  correspond 
to  certain  changes  in  the 
skin  found  in  acquired 
sypliilis,  the  former  give  to 
the  child  a  characteristic 
appearance  which  manifests 
itself  primarily  in  the  con- 
sistenc)-  of  the  skin  of  the 
face.  I  ha^'c  called  tliis 
change  cUft'use,  superficial, 
sypliihde,  or  diffuse  hered- 
itary-sypMhtic  skin  infiltra- 
tion. Soon  after  the  appearance  of  the  nasal  symptoms  the  skin  of  the 
face  assumes  a  peculiar,  pale,  yellow  tint,  and  is  somewhat  glossy, 
symptoms  that  depend  not  so  much  upon  insufficient  blood  suppl}-,  as 
upon  a  mild  infiltration  of  the  papillary  portion  of  the  skin  and  upon 
increased  tension  in  the  rete  of  Malpighi. 

The  color  resembles  at  first  a  pale  caf6  au  lait,  after  a  longer  period 
of  time  when  more  pigmentation  has  taken  place,  the  color  of  the  finger 
of  a  cigarette  smoker.  These  changes  are  especially  marked  on  the 
cheeks  and  on  the  cliin,  but  also  appear  like  spectacle  rims  on  the  orbital 
borders,  or  like  the  expanded  wings  of  a  butterfly  about  the  root  of  the 
nose,  or  like  a  gotee  on  the  under  hp. 

A  diffuse  infiltration  of  the  borders  of  the  Ups  is  very  character- 
istic. This  produces  a  pecuUar  stiffness,  a  brownish  red  color,  and  a 
striking  glossiness  (Fig.  117).    Soon  radial  fissures  and  rhagades  appear 


Fig.  117. 

91 

0 

^^^^Bi-V^  'rZJMK 

0H 

n«v 

Macular  syphilides  of  the  skin  of  the  face  with  a  liig:h 
degree  of  diffuse  infiltration  of  the  borders  of  the  lips  in  a  child 
five  weeks  old. 


SYPHILIS  515 

in  the  infiltrated  skin  areas  in  those  places  where  muscular  action  keeps 
the  skin  in  motion,  as  about  the  mouth  and  nostrils,  and  on  the  eyelids. 
Similar  infiltrations  affect  the  hairy  scalp  leading  to  loss  of  hair,  and 
also  with  great  partiaUty,  the  skin  of  the  flexor  surfaces  of  the  lower 
half  of  the  body  and  that  of  the  genito-anal  region. 

External  irritants  e.xert  an  undeniable  influence  upon  the  production 
of  this  form  of  syphihs.  Tliis  accounts  for  the  predilection  for  the  lower 
lialf  of  the  body,  which  is  constantly  exposed  to  the  irritating  effect  of 
feces  and  urine.  Not  rarely  one  sees  in  congenitally  syphilitic  infants 
during  the  eruptive  stage  the  conversion  of  an  intertriginous  skin  affec- 
tion into  a  diffuse  superficial  syphilide,  with  a  change  from  a  light  red, 
oozing  skin  to  one  that  is  brownish  and  has  a  peculiar  stiffness,  drj'ness, 
and  glossiness.  Frequentlj'  the  skin  infiltration  is  localized  on  the  flexor 
surfaces  of  the  lower  extremities  like  the  leather  portion  of  a  pair  of 
riding  breeches. 

Independently  of  external  macerating  influences,  the  skin  of  the 
soles  of  the  feet  and  of  the  palms  of  the  hands  is  always  involved  at  the 
very  first  in  a  diffuse  manner,  on  account  of  the  early  and  very  abundant 
development  of  sweat  glands  in  those  regions.  The  skin  becomes  hard, 
smooth,  and  free  from  wrinkles  and  glossy  as  if  varnished  or  painted  with 
water  glass,  with  a  color  that  at  first  is  reddish  yellow  later  brownish,  or 
salmon  colored.  Aery  frequently  diffuse  involvement  of  the  skin  of  the 
soles,  palms  and  face,  is  a  forerunner  of  the  appearance  of  regular, 
circumscribed  exanthemata,  frequentlj',  however,  it  forms  the  only 
cutaneous  lesion. 

Diffuse  hereditary-syphiUtic  skin  infiltration  may  be  di\'ided  into 
three  forms,  or  stages,  between  wliich  transitional  forms  exist. 

1.  Diffuse  smooth  infiltration,  or  erj'thematosa  simplex.  This  is 
frequent  on  the  soles  and  palms,  but  also  on  the  chin,  on  the  glabella, 
on  the  preauricular  hairy  portions  and  about  the  neck.  The  color  of  the 
smooth  scaleless  skin  that  is  involved  may  show  all  kinds  of  tints  from 
a  light  cherry  red  to  the  darkest  blue  red  (Plate  27). 

2.  Diffuse,  desquamative,  or  lamellar  infiltration.  In  this  the 
horny  layers  of  the  skin  are  loosened  and  separated  in  large  lamellse, 
or  masses,  wMle  the  texture  of  the  skin  appears  sclerosed  and  very 
much  thickened  (Plate  24,  III). 

3.  Eroded  infiltration.  Tliis  term  apphes  to  all  ulcerated,  oozing, 
moist,  and  impetiginous  forms. 

Tills  diffuse  specific  skin  infiltration  can  arise  under  many  difTerent 
conditions:  (1)  by  confluence  of  a  number  of  disc-hke  areas  the  size  of  a 
penny  to  that  of  a  dollar,  of  pale  rose  color,  not  raised  above  the  general 
surface  of  the  slcin;  (2)  on  top  of  a  diffuse  uniform  erythema;  (3)  by  the 
rapid  confluence  of  very  rapidly  arising,  small,  pale  red,  closely  packed, 
individual  efflorescences;  (4)  by  the  confluence  of  real  lenticular  papules. 


516 


THE   DISEASES   OF   CHILDREN 


Tliis  diffuse  hereditary-syphilitic  lesion  is  most  frequently  found 
during  the  first  three  months  of  hfe  and,  according  to  our  investigations, 
is  never  present  at  birth.  It  is  a  very  frequent,  but  by  no  means  con- 
stant slcin  affection  of  hereditary  sypliilis  that  ushers  in  the  jieriod  of 
cutaneous  manifestations,  but  can  also  reappear  at  any  time  during  the 
first  year  as  a  recurrence. 

A  special  form  of  this  diffuse  skin  infiltration  in  hereditarj'  syphi- 
hs  is  found  in  specific  paronychia,  which  is  accompanied  by  trophic  dis- 
turbances of  the  nails  (see  Plate  24).  Two  forms  are  distinguishable: 
paronychia  sicca,  and  paronychia  ulcerosa.  The  skin  adjacent  to  the 
bases  of  the  nails  of  both  fingers  and  toes  appears  brownish  red,  thick- 


Fio.   US. 


Diffuse  crusty  syphilide  of  tlie  face.     Myotonia  spa^^tica. 


ened  and  glossy,  and  covered  with  scales,  or  with  crusts.  As  soon  as 
tliis  specific  involvement  of  the  matrix  of  the  nail  has  persisted  for  some 
time,  it  is  noticed  that  the  nail  is  divided  by  a  whitish  transverse  furrow 
into  two  parts  which  seem  separate  from  one  another  and  have  very 
different  characteristics.  The  proximal  portion  is  thinner  and  marked 
by  longitudinal  furrows  wliile  the  distal  portion  appears  normal,  "  or  at 
least  a  somewhat  increased  brittleness."  Very  frequently  the  nails  are 
completely  softened  or  disintegrated. 

Tliis  diffuse  sypliihde  of  infancy  has  a  special  predilection  for  the 
hairy  scalp.  Whenever  a  hairy  portion  of  the  body  is  involved  this 
leads  at  first  to  a  marked  increase  in  desquamation  and  in  sebaceous 
secretion.  Masses  of  sebum  are  rapidly  formed — much  more  rapidly 
than  in  a  simple  nonspecific  seborrhoea. 

The  .scalp  secretion  always  becomes  thicker  and  tougher,  it  rapidly 


SYPHILIS 


517 


dries  up  wth  the  scales  into  a  hard- crusty  mass  that  covers  the  head 
completel}-  like  a  hood.  These  masses  do  not,  however,  as  a  rule  show 
the  straw  yellow  color  of  tlie  crusts  of  simple  seborrhrra,  but  rather  are 
Ught  brown,  with  a  base  of  in-  Fig.  u9, 

filtrated,  copper-colored  skin, 
not  one  that  is  bright  red, 
swollen  and  oozing.  At  the 
same  time  the  scales  are  always 
less  firmly  united  to  the  skin 
thanineczemawith  crusts,  and 
can  usually  be  picked  off  with- 
out causing  bleeding. 

It  is  also  very  significant 
that  the  affection  almost  never 
moistens  the  scalp,  as  opposed 
to  the  condition  in  eczema. 
Under  these  masses  of  sebum 
there  is  usually  found  in  these 
cases  of  diffuse  syphiUde  of  the 
scalp,  a  perfectly  intact  epidermis,  while  in  seborrhceal  infantile  eczema 
of   the   scalp,  when    the  crusts  are   Hfted,  the   bared  rete   Malpighii  is 

Fig.  120. 


Syphilitic  pemphigus  of  the  newljuni  un  the  soles  of 
the  feet  in  a  child  seven  days  old.  The  skin  infiltrated 
in  toto.    Pemphigus  biebs  broken. 


e        c    c  c 

Vertical  section  of  a  syphilitic  pemphigus  bleb  on  a  diffusely  infiltrated  plantar  skin  area.  (a>  rete  Mal- 
pigliii,  infiltrated  with  round  cells  (f/i;  (h)  horny  layer  torn  and  lifted  up  in  a  number  of  layers;  (.6)  coriuni 
witli  proliferated  connective  tissue  cells;  (c)  sweat  glands;  (d)  glantl  tubules  ending  in  the  papillary  layer 
without  connection  with  the  epidermis;  (e)  a  section  of  blood  vessel  with  pe^i^'ascula^  granulations;  (/)  greatly 
infiltrated  and  swollen  papillary  portion;  between  a  and  /  is  a  close  space  resulting  from  the  separation  of 
the  rete  Malpighii  from  the  papillary  layer;    (P)  pemphigus  bleb.     (Slight  magnification.) 

exposed,  or  if  still  more  intensely  inflamed,  the  bleeding  papillary  layer. 

This  same  process  manifests  itself  in  a  very  similar  manner  in  the 

region  of  the  eyebrows.      In  many  cases  an  early  dififuse  involvement 


518 


THE   DISEASES   OF   CHILDREN 


of  all  the  hairy  regions  leads  to  complete  alopecia.  The  characteristic 
absence  of  hair  on  the  scalp  and  on  the  eye-brows  and  eye-lashes  in  older 
infants  afflicted  with  hereditary  syplilHs  is  explained  in  this  same  way. 
Occasionally  there  is  a  facial  eczema  implanted  upon  the  diffusely 
infiltrated  skin  of  hereditary  syphilis  (Fig.  118).  On  the  borders  of  the 
eyeUds,  the  nostrils,  and  the  lips,  the  infiltrated  skin  easily  cracks  and 
so  leads  to  crust  formation.  Apart  from  the  rhagades  the  whole  skin  of 
the  face  may  be  covered  with  reddish  brown  or  brownish  yellow  crusts. 
In  severe  cases  a  rupiaform  syphiUdc  results. 

The    Circumscribed    Exanthemata    of    Early    Hereditary    Syph- 
ilis.— These   appear   either   upon    a   diffuse   skin  infiltration   or   upon 
Fig.  121.  a  prcviously  unaltered  skin. 

Pew? phigus  Syph  iliticus 
Neojiatorum. — In  the  major- 
ity of  cases  tliis  is  present 
at  birth,  occasionally  it  ap- 
pears during  the  first  week, 
rarely  during  the  second  to 
the  fourth  week.  If  present 
at  birth,  or  occurring  during 
the  first  few  days,  it  has  an 
ominous  significance,  less  so 
if  it  occurs  later.  Essentially 
there  is  no  difference  between 
these  two  forms  of  pem- 
pliigus,  wliich  would  be  more 
jiroperly  designated  by  the 
terms  papulo- bullous,  or 
papulo  -  pustular,  sypliiUde. 
In  pemphigus  of  the  newborn 
we  have  an  eruption  com- 
posed of  vesicles  or  blebs,  varying  in  size  from  that  of  a  pea  to  that  of  a 
penny,  filled  with  purulent  or  bloody  fluid,  and  located  on  disc  shaped 
brownish  red  spots.  The  blebs  are  surrounded  by  an  infiltrated  border. 
They  always  appear  first  on  the  soles  of  the  feet  and  on  the  palms  of  the 
hands,  but  can  later  invade  other  regions  of  the  body,  not,  however, 
without  being  preceded  by  an  infiltration  of  the  skin  (Figs.  119  and 
120;  and  Plate  26).  As  a  result  of  the  confluence  of  several  blebs  and 
the  destruction  of  their  roofs,  ulceration  takes  place,  and  in  rare  cases 
circumscribed  gangrene  of  the  skin  may  supervene.  Streptococci  and 
staphylococci  are  found  in  great  numbers  in  the  secretion  within  the  blebs. 
Schaudinn's  spirocha'te  paUida,  too,  has  recently  been  found  repeatedly. 
The  form  of  eruption  that  I  have  designated  as  late  pemphigus  has 
the  same  localization  as  the  early  form  which  is  present  at  birth,  or 


Isolated    papules  on   the   fcieheail.     Hydrocephalus   in 
miniature.    Saddle  nose. 


SYPHILIS  519 

within  a  few  days  after  birth,  but  appeals  more  frequently  superimposed 
on  an  area  of  diffuse  skin  infiltration  (see  Plate  28). 

Histologically  there  is  in  both  forms  a  uniform  inflammatory  cell 
proliferation  of  the  papillary  portion,  follomng  the  blood  vessels.  There 
is  a  striking  broadening  and  a  serous  iniliibition,  on  the  part  of  the 
papilla>  and  a  separating  from  them  of  the  rete  nmcosum,  so  that  within 
the  area  covered  by  the  bleb  there  is  a  complete  denudation  of  the 
papilla?  of  their  epidermal  covering  (see  Fig.  120).  At  the  same  time 
there  occurs  sphtting  of  the  hornj-  layer  itself. 

The  remaining  early  exanthemata  of  hereditary  syphilis  differ 
but  httle  from  those  of  acquired  syphiHs.  The  following  forms  of  erup- 
tion may  be  distinguished: 

1.   Maculopapidar  Syphilide. — (Figs.  117,  121,  122  and   Plate  26). 

This  occurs  as  a  rule  after  a  period  of  incubation  lasting  several 
weeks,  and  consists  of  more  or  less  numerous  disc-shaped  spots,  slightly 
raised  above  the  general  skin  level,  varying  in  color  from  an  early  rose 
red,  to  a  later  brownish,  or  ham  colored,  or  after  persisting  for  some 
time,  to  an  ochre  yellow.  The  locations  of  predilection  are  the  lower 
extremities,  the  flexor  surfaces  of  the  upper  extremities,  the  neck,  cliin 
and  face.  Palms  and  soles,  too,  are  frecjuently  affected.  The  papules 
may  be  absorbed  from  the  centre  leaving  pigmented  spots,  or  they  may 
undergo  desquamation,  or  in  the  presence  of  mechanical  irritation  they 
may  grow  extensively  in  all  directions,  and  form  the  so-called  condy- 
lomata lata.  The  latter  never  appear  as  the  first  eruption  of  congenital 
syphihs,  but  are  always  an  expression  of  a  relapse.  They  occur  usually 
in  places  where  two  oppo.sing  skin  surfaces  rub  together  as  in  the  cir- 
cumanal and  genitocrural  regions,  in  the  interdigital  folds  and  about 
the  navel.  They  are  easily  eroded  and  then  show  a  lardy  yellow,  or 
diphtheroid,  surface.  When  the  secretion  ceases  and  the  condylomata 
dry  up,  the  color  becomes  lighter,  the  centres  become  depressed  and  the 
growths  are  covered  with  a  la3'erasif  thej'had  been  painted  with  collodion. 

In  early  congenital  syphilis  the  roseola  of  the  acqiured  type  is 
never  found.  Likewise  the  trunk  is  usually  whoUj-  free  from  eruption 
in  the  congenital  variety. 

The  papular  eruption  may  be  the  first  exanthem  in  congenital  syph- 
ihs or  may  represent  a  relapse  of  the  disease.  In  the  latter  case  it  has 
been  preceded  either  by  diffuse  skin  affections,  or  simply  by  A-isceral 
and  osseous  manifestations. 

Occasionally  vesicles  and  jnistules  arise  from  broad  papules  by 
elevation  of  the  epidermis. 

The  favorite  location  of  maculopapular  eruptions  is  the  forehead 
and  the  hairy  scalp,  usually  after  the  dift'use  skin  infiltration  has  pre- 
ceded it.  A  dense  crown  of  papules  is  frequently  formed  on  the  fore- 
head in  relapses  (see  Plate  27). 


580 


THE   DISEASES   OF   CHILDREN 


Most  of  the  papules  disappear  ultimately  by  simple  absorption, 
those  that  are  larger  and  more  elevated  after  a  preceding  desquama- 
tion, the  oozing  forms  after  a  preceding  crust  formation. 

When  crust  formation  has  taken  place  extensively  on  the  face  and 
on  the  hairy  scalp,  there  is  an  appearance  that  greatly  resembles  the 
stages  of  crust  formation  in  impetiginous  eczema.  But  the  brownish 
yellow  borders  of  the  infiltrate  with  their  glued  appearance,  as  well  as 
the  crust-free  infiltrated  skin  areas  ^ith  their  almost  metallic  lustre, 
the  peculiar  stiff,  reactionless  character  of  the  whole  inflammatorj'  pic- 
ture, and  the  absence  of  serous  discharge  between  the  scabs  point  to  the 
fact  that  back  of  this  crust  formation  there  is  not  an  eczema,  but  a 
syphiUtic  infiltration. 

2.  Papulopustidar  Sypltilide. — Tliis  form  of  eruption  has  a  rela- 
tively short  incubation  period.     Pustules  with  tliin  purulent  contents. 


Fia.  122. 


Maculopapular  syphilide  of  the  skin  of  the  face  and   extremities.    Trunk  free.    Nose  sunken  in.     Rhagadic 
ulcers  on  the  Infiltrated  borders  of  the  lips.    Myotonia  perstans. 


not  unlike  the  pustules  of  smallpox,  appear  on  deep  red  flattened 
papules  about  the  size  of  a  lentil.  Usually  they  appear  only  as  scattered 
le.sions.  Through  drying  of  the  contents  of  the  pustule  cupshaped  crusts 
frecjuently  are  formed  (rupiaform  syplrilide).  Freciuently  deeper  ulcer- 
ation takes  place  with  the  formation  of  crusts  that  resemble  oj'ster 
shells  (ecthyma  syphiliticus).  These  forms  belong  to  the  severe  mani- 
festations of  infantile  sj-phihs  and  give  an  unfavorable  prognosis.  One 
must  not  confuse  with  these  pustular  sypliihdes  secondary  septic,  or 
pysemic  skin  lesions  in  children  that  have  hereditary  syphilis. 

.3.  Ulcerative  Syphilide. — In  locations  that  are  naturally  exposed 
to  maceration  and  are  not  kept  clean,  ulcers  are  formed  through  the 
destruction  of  papules.  These  are  characterized  by  a  pecuhar  basin- 
Uke,  sunken  surface,  by  a  dry,  shining  coat,  and  by  its  infiltration 
wall  and  absence  of  reaction.  The  site  of  predilection  is  the  genito- 
anal  region.  But  in  other  regions  too  papules  may  become  eroded  and 
ulcerated  when  exposed  to  mechanical  and  chemical  irritation.  Fur- 
thermore, all  forms  of  vesicular  and  pustular  syphilides  may  lead  to 


SYPHILIS 


521 


Fig.  123. 


ulceration.  These  ulcers  are  all  distinguished  from  nonsyphilitic 
ulcers  bj-  the  absence  of  inflammatory  reaction,  by  the  small  amount 
of  pain,  by  the  presence  of  peculiar,  dry,  firmly  adherent  crusts  and 
by  their  central  depression.  Probably  all  pustular  and  ulcerative 
sypliilides  depend  upon  a  double  infection,  i.e.,  with  the  specific  germ  of 
syphihs  and  with  pyogenic  liacteria. 

4.  Sniall  Papular  SyphiHde. — This  is  extremely  rare  in  infants 
and  according  to  my  observation 
occurs  only  as  a  manifestation  of 
a  relapse  in  hereditary  syphihs 
during  the  second  half  of  the  first 
year.  It  greatly  resembles  tul)ercle 
of  the  skin  (tul^ercuhde.^),  but  is 
distinguished  by  the  brownish 
color,  the  pecuUar  glossiness,  and 
the  hardness  of  the  indi^'idual 
papule.  I  have  seen  it  on  the  nape 
of  the  neck,  the  back,  the  forehead, 
and  on  the  extremities,  partly  scat- 
tered, partly  arranged  in  groui^s. 
At  most  the  papules  are  l)ut  few 
in  number.  Exceptionally  I  have 
.seen  this  form  of  eruption  arise  as 
late  as  the  second  or  tWrd  year. 
This  syphilide  is  verj'  intractable 
from  a  therapeutic  j<tan<lpoint. 

Lesion  of  the  Mucous  Mem- 
branes.— Apart  from  nasal  affec- 
tions that  have  already  been  dis- 
cussed, involvement  of  the  mucous 
membranes  in  hereditary  sypliihs 
during  infancy  is  a  rare  occurrence. 
Diffuse  infiltration  and  papule 
formation,  it  is  true,  favor,  as 
already  indicated,  the  regions  about 
the  openings  into  the  body,  thus 

bordering  on  th^e  adjacent  mucous  membranes  without  however  invading 
the  latter.  Occasionally  a  stubborn  hoarseness  amounting  even  to 
aphonia  indicates  an  involvement  of  the  laryngeal  mucous  membranes, 
but  only  exceptionally  during  or  soon  after  the  first  eruption.  Usually 
tliis  is  one  symptom  among  others  of  a  relapse  in  wliich,  as  a  result 
of  oedema  of  the  glottis  and  perichondritis,  severe  attacks  of  suffocation 
can  occur  that  may  even  necessitate  tracheotomy. 

In   spite   of  the  intimate   connections   between   the   nasal   mucous 


Papulopustular  s^•pllillde  (late  pemphigus). 
Child  of  fuur  weeks.  Lesions  chiefly  on  tiie  extrem- 
ities, more  especially  the  lower  ones,  .\mong  simple 
papules  others  that  are  surmounted  with  vesicles.  In 
addition  osteochondritis  of  the  left  humerus  and  ulna 
with  swelling  of  atTected  joints  and  flaccid  paralysis. 


522 


THE   DISEASES   OF   CHILDREN 


Fir..  124. 


Fen,  ur 


membrane  and  that  of  the  middle  ear,  and  in  spite  of  the  rather  frequent 
attacks  of  middle  ear  disease  in  infancy,  discharge  from  the  ear  is  a  rare 
affection  in  early  hereditary  syphihs. 

A  typical  involvement  of  a  mucous  membrane  is  to  be  found  in 
Mracek's  syphilis  anmdaris  intestini  localized  in  Peyer's  patches.  This, 
together  with  diffuse  thickenings  of  the  gastro-intestinal  mucous  mem- 
brane that  are  a  result  of  inflammatory  cell  infil- 
tration areas  accompanied  by  partial  disappearance 
of  the  glandular  elements,  is  perhaps  the  cause  of 
intestinal  disturbances  in  infants  with  congential 
syphihs,  although  other  factors  doubtless  play  a  part. 
In  children  that  are  born  healthy  the  first 
evidence  of  syphilis  is  nearly  always  to  be  found  in 
symptoms  of  disturbed  general  health,  with  nervous 
unrest,  increased  tension  in  the  fontanelle,  rise  of 
temperature,  and  deficient  gain  in  weight.  The 
appearance  of  diffuse  skin  infiltration  and  papular 
eruption  is  accompanied  in  my  experience  by  only 
slight  rise  of  temperature,  while  the  eruption  of 
pustular  sypliiUdes  always  brings  out  a  temperature 
up  to  39°  C.  (102°  F.)  lasting  for  a  number  of  days, 
and  frequently  lasting  many  days  longer  than  the 
eruption  itself,  probably  due  to  a  mixed  infection. 
Changes  in  the  blood  picture  are  always  found 
soon  after  the  eruption  begins,  but  very  frequently 
also  before  that  time.  They  consist  in  a  diminution 
in  the  amount  of  hirmoglobin  and  in  the  number  of 
erythrocytes  together  with  the  appearance  of  many 
nucleated  red  blood  corpuscles  and  an  increase  in 
the  number  of  white  blood  cells,  especially  the 
myelocytes  and  eosinopliiles. 

From  a  diagnostic  standpoint  it  must  be  remem- 
bered that  lesions  of  the  skin  and  of  the  mucous 
membranes  may  be  very  insignificant  and  that  the 
diagnosis  of  early  hereditary  sypliihs  must  not 
depend  upon  the  presence  or  absence  of  skin  lesions. 
Bone  Lesions  in  Early  Hereditary  Syphilis. — The  osseous  system 
is  involved  at  least  as  frequently  as  the  skin  in  hereditary  sypliihs. 
Involvement  of  the  bones  that  are  preformed  in  cartilage  nearly  always 
begins  in  intra-uterine  hfe,  while  that  of  the  bones  that  form  in  mem- 
branes usually  occurs  after  birth.  In  fact  Wegner's  osteochondritis 
which  occurs  at  the  epiphyseal  borders  of  the  hollow  bones  is  a  foetal 
manifestation,  wliile  the  hyperostoses  of  the  cranial  bones  usually  do 
not  arise  until  after  birth. 


Sagittal  section  of  the 
knee-joint  of  amacerated 
foetus  with  hereditary 
syphilis  in  the  la«t  lunar 
month  of  pregnancy. 
Werner's  osteochon- 
dritis, second  stage.  A. — 
Broadened  irregular  zone 
of  calcification  with  .ser- 
rated border;  long  pro- 
jections into  the  hyaline 
cartilage.  B. — Very  wide 
medullary  space  with 
granulation  tissue.  C. — 
Hypera-mic  layer  of 
spongiosa,  adjacent  t  o 
the  medullary  space. 


PLATE  26. 


SYPHILIS 


523 


The  explanation  for  the  innate  relationship  between  the  poison  of 
congenital  sypliilis  and  the  fa?tal  osseous  system  is  to  be  found  in  the 


Fic.  12.';, 


Fig.  126. 


Rontgen  picture  of  the  upper  extremity  of  a  fcetus  bom  dead  from  hereditary  sMjhiHs.  At  the  proximal 
end  of  the  humerus  and  at  the  lower  ends  of  the  diaphysps  of  tlie  forearm  marked  broadening  of  the  zone  of 
calcification  with  pointed  projections  toward  the  epiphj'ses.  At  the  distal  end  of  the  upper  arm  behind  the 
rather  strongly  rarified  zone  of  calcification  well  marked  lightening  of  the  diaphyseal  shadow.     (Natural  size.) 

powerful  and  peculiar  method  of  growth  of  the  skeleton,  wliich  has  this 
peculiarily,  that  it  grows,  not  uniformly,  but  by  apposition  either 
through  metaplasia  of  cartilage  or  through  increase  from  the  periosteum. 
Those  portions  of  the  bony 
system  in  which  growth  is 
most  active  are  the  ones  that 
most  attract  the  poison  of 
sypliilis  circulating  in  the 
foetal  organism,  and  so  there 
arises  in  ftrtuses  and  young 
infants  that  affinity  for  the 
epiphyseal  borders  of  the  long 
hollow  bones  and  for  the 
tuberosities  of  the  cranial 
bones.  In  this  so-called 
osteochondritisheredosyphil- 
itica  of  Wegner  the  most 
proininent  changes  that  are 
found  are  disturbances  in 
the  metaplasia  of  the  car- 
tilaginous ground  substance 
about  the  i  n  tracliondral 
medullary  spaces,  abnormal 
proliferations  of  cartilage 
cells,     necrotic     processes 


within    the   cartilage,   intra- 


Rontgen  picture  of  the  lower  extremity  of  a  SMihilitic 
newborn  child.  Broadening  of  tlie  zone  of  calcification  with 
a  ragged  e^ige  toward  the  epiph>seal  cartilage.     tNatural  .size.) 


chondral  cleft  formation,  and 
pathological  calcification. 

In  the  zone  of  the  subchondral  medullary  space  formation  there 
occur  at  first  hypersemic  inflammator}-  processes  with  simultaneous 
transformation   of   the   marrow  of  the  .spongy  portion  into  granulation 


524  THE    DISEASES   OF   CHILDREN 

tissue  and  the  failure  of  osteoblast  formation.  Frequently  before  this 
time  changes  in  the  consistency  of  the  marrow  have  appeared  at  the 
diaphyseal  ends  with  retrogression  of  vascularization.  The  marrow  at 
the  cartilaginous  borders  often  becomes  transformed  into  fibrous  con- 
nective tissue. 

The  disturbances  of  periosteal  and  perichondral  ossification  con- 
sist at  first  of  subperiosteal  envelopment  of  granulation  tissue,  from 
wliich  results  a  melting,  as  it  were,  of  the  compact  bony  substances. 
Through  calcification  of  tliis  granulation  tissue  there  arises  abnormal 
hyperostosis,  often  even  the  formation  of  numy  laj^ers  about  the  pri- 
mary bone  which  is  embedded  in  the  new  shell  of  bone,  hke  the  end  of 
a  cigarette  in  its  holder.  Besides  this  ossifying  periostitis  there  is  also  a 
chondrifying  periostitis  in  the  region  of  the  epiphyseal  borders,  if  witliin 
these  there  is  taking  place,  or  has  taken  place,  a  break  in  continuity. 
Under  these  conditions  there  can  occur,  just  as  in  real  fractures,  the 
formation  of  genuine  callus,  at  first  cartilaginous,  later  bony. 

According  to  Wegner  (1870)  three  stages  of  osteochondritis  can  be 
distinguished.  In  the  first  stage  the  zone  of  calcification  of  the  car- 
tilage is  broadened  and  irregular,  distinguished  from  the  bluish  shim- 
mering cartilage  and  from  the  strongly  hypersemic  spongiosa  by  its 
greater  density  and  fighter  color.  The  second  stage  is  characterized  by 
the  estabUshment  of  a  broad  mortar-hke  layer  belonging  to  the  pro- 
visional zone  of  calcification  between  the  epiphyseal  cartilage  and  the 
diaphysis.  In  the  tliird  stage  there  follows  in  connection  with  the 
hyafine  cartilage  toward  the  diaphyses  a  whoUj'  irregularly  defined 
layer  several  millimetres  wide  of  a  grayish  yellow  mortar-Hke  mass  which 
is  very  compact  (broadened,  irregular  calcification  of  cartilage).  Next 
to  tliis  is  a  grayish  yellow  or  grayish  red  layer  of  varjing  width  and  of 
fight  density,  that  gradually  disappears  as  the  spongiosa  is  approached. 
On  account  of  this  mass  of  sfight  resistance  the  natural  connection  be- 
tween epiphysis  and  diaphysis  is  disturbed,  frequently  to  such  an  extent 
that  the  shaft  of  the  bone  remains  attached  to  the  epiphysis  only  through 
the  thickened  periosteum. 

The  beginning  stages  of  osteochondritis  are  u.sually  not  recogniz- 
ble  macroscopically,  but  can  be  demonstrated  microscopicaUy. 

With  reference  to  the  anatomical  conditions  in  separation  of  the 
epiphyses  in  hereditary  syphihs,  one  must  remember  that  tliis  is  essen- 
tially a  fracture  wliich  takes  place  either  in  the  subchondral  granulation 
tissue,or,if  necrotic  processesexist,  in  the  columnar  portion  of  the  cartilage. 
Lessening  of  density,  trauma,  and  muscular  action,  are  the  causal  factors. 
For  this  reason  epiphyseal  separation  in  hereditary  syphihs  occurs 
only  in  the  long  hollow  bones,  never  in  the  ribs,  or  short  hollow  bones. 

The  short  hollow  bones  show  intrachondral  and  periosteal  patho- 
logical processes  that  are  quite  similar  to  those  found  in  the  long  bones. 


SYPHILIS 


525 


526 


THE   DISEASES   OF   CHILDREN 


There  are  disturbances  in  the  endochondral  ossification  and  rarifying 
diaphyseal  processes,  and  also  characteristic  periosteal  affections.  Sepa- 
ration of  the  epiphyses  and  involvement  of  the  small  joints  are  never  found. 
Joint  affections  are  very  rare  in  hereditary  sypliihs  during  the 
first  year,  but  they  do  occur  during  the  second  year,  especially  in  the 
larger  extremities,  but  only  rarely  in  those  connected  mth  the  verte- 
bral  column.     Joint   suppuration   in   sypliilitic   infants  is   always  the 

Fig.  128. 


Fig.  129. 


:i 


Semidiagrammatic  Rontgen  picture  of  the  right 
hand  of  a  three  and  one-half  months  old  child,  with 
epiphyses  of  the  forearm  formerly  separated  and 
poorly  united.  Pathological  inflammatory  calcifi- 
cation of  the  epiphyseal  cartilage.  Also  multiple 
involvement  of  the  short  hoUow  bones. 


Semidiagrammatic  Rontgen  picture  of  the  left 
upper  extremity  of  a  child  two  and  one-lialf  months 
old  with  general  involvement  of  the  whole  osseous 
system  and  pseudoparalysis  of  both  upper  extremi- 
ties. Phalangitis,  endosteal  and  periosteal  disease  of 
the  long  hollow  bones.  A  . — Rarefying  osteitis  of  the 
sliort  bones  with  swelling  and  faint  shadow.  B. — 
Diffuse  periosteal  osteophyte  of  the  bone  of  the 
forearm.  C. — Swelling  and  ilarefaction  at  the 
diaphyseal  ends. 


result  of  mixed  infection.  The  confounding  of  gonorrhoeal  and  pyiemic 
joint  inflammations  with  osteochondritis  of  congeni tally  sypliihtic 
origin  has  doubtless  occurred  many  times,  especially  since  the  extremities 
are  found  in  positions  that  suggest  paralysis  in  all  of  these  conditions. 

Radioscopy  is  of  great  importance  in  hereditary  sypluhtic  lesions 
of  the  osseous  system. 

Sypliihtic  osteochondritis  is  demonstrable  in  X-ray  pictures  of  older 
foetuses  and  widening  of  the  zone  of  calcification  "vvith  its  irregular 
jagged  border  can  regularly  be  made  out  in  dead  specific  foetuses  (Figs. 


SYPHILIS 


527 


125  and  126).  In  living  infants  too,  with  syphilitic  infection,  if  kept 
absolutely  quiet,  the  presence  of  general  affections  of  the  osseous  system 
of  osteochondral  or  periosteal  nature  in  the  long  and  short  hollow 
bones,  wliich  have  not  caused  chnical  symptoms  can  frequently  be 
discovered.  The  so-called  pseudoparalysis  heredosypliihtica  (see  later) 
has   always   shown,  fig.  i3o. 

in  those  cases  I  have 
examined  \nth  the 
X-ray,  recognizable 
changes  in  the  os- 
seous system,  which 
have  consisted  in 
lessening  of  the  dia- 
phj'seal  shadow,  or 
in  periosteal  hyper- 
ostosis, or  in  swell- 
ing of  the  bone  (Figs. 
127  and  128).  In 
genuine  separation 
of  the  epiphysis 
there  is  shown  a 
periostealinflamma- 
tory  lime  deposit  at 
the  diaphyseal  bor- 
der wliich  extends 
over  to  the  epiphysis 
and  may  produce 
very  irregulai 
shadow  effects  of 
the  latter. 

Affections  of 
the  Short  Hollow 
Bones. — The  hered- 
itary  syphilitic 
lesions  of  the  bones 
of  the  fingers  and 
toes  in  infants  were 
only  rarely  described 

before  my  own  investigations  were  made.  Specific  involvement  of 
the  phalanges,  which  occurs  much  more  frequently  in  infancy  in  the 
fingers  than  in  the  toes,  affects  only  the  bones  of  the  phalanges,  never 
the  soft  parts  and  always  begins  in  the  proximal  phalanges.  The  latter 
are  also  more  intensely  affected  in  the  further  course  of  the  disease 
than  are   the   distal   phalanges. 


Longitudinal  section  throuph  the  tibialis  posticus  muscle  below  the 
upper  tibial  epiphysis  in  a  congenitally  sj-philitic  child  of  2  months,  together 
with  the  periosteum  and  periosteal  inflammatory  proliferation.  O. — <?al- 
cified  osteoid  originating  in  the  inflamed  periosteum.  K.K. — Cartilage 
callus,  chondroid  proliferation  within  newly  formed  bone  resulting  from 
inflamed  periosteum.  Pe. — Swollen  and  cedematous  fibrous  layer  of  the 
periosteum  with  spaces  between  the  bundles  of  elastic  fibre.s.  Ik. — Focus 
of  infiltration  of  round  cells  extending  from  the  inflamed  periosteum 
toward  the  muscle;  the  sarcolemma  of  the  latter  is  much  disintegrated. 
Bg. — Blood  yessels  with  peri\ascular  inflammation  and  proliferation  of 
intima.  .1/e.— Inflamed  and  disintegrated  bundles  of  muscle  fibres,  cut  longi- 
tudinally and  diagonally,  with  infiltrated  perimysium  (Pm^.  .1/r.— Remnant 
of  muscle  tissue  little  changed.    iV. — Cross  section  of  normal  new  fibre. 


528  THE   DISEASES   OF   CHILDREN 

The  X-raj'  i)ictiii-e  of  tlie  diseased  phalanges  shows  three  degrees 
of  shadow:  a  moderate  lessening  of  depth  of  shadow  of  the  epiphyseal 
borders,  a  still  greater  one  of  the  diaphyses  and  a  dark  faint  shadow; 
but  a  sharply  defined  marginal  shadow  corresponds  to  the  compact 
portion  of  the  bones.  At  the  same  time  tlie  bone  seems  abnormally 
swollen  both  as  to  width  and  length  (Fig.  129).  All  of  this  points  unde- 
niably to  the  fact  that  in  phalangitis  there  is,  from  a  pathological  stand- 
point, a  diffuse  rarefying  osteitis  of  the  phalangeal  bones,  that  occurs 
much  more  frequently  than  is  usually  thought  to  ho  tlie  case  from  mere 
chnical  examination. 

From  a  clinical  and  diagnostic  standjjoint  the  following  facts  are 
important:  the  predominating  involvement  of  the  basal  phalanx,  the 
absence  of  suppuration  or  of  external  jierforation,  the  tendency  to 
spontaneous  restitution  and  the  subacute  course  of  the  disease.  This 
painless  swelhng,  involving  first  the  proximal  phalanx,  and  always  the 
bone  only,  gives  to  the  finger  the  form  of  a  bottle:  with  simultaneous 
involvement  of  the  distal  phalanges  it  takes  the  form  of  a  tenpin.  The 
finger  always  appears  broader  as  well  as  longer.  The  soft  parts  do  not 
take  part  in  the  disease  but  the  skin,  on  account  of  stretcliing,  may  be- 
come glossy,  tense  and  peculiarly  rosy — sometimes  it  may  even  appear 
tliinned.  The  index  finger  is  most  frequently  affected.  These  lesions 
tend  to  multipHcity  but  not  to  symmetry.  A  further  characteristic 
lies  in  the  complete  absence  of  involvement  of  the  joints  adjacent  to 
the  diseased  phalanges.  This  affection  belongs  to  the  early  manifes- 
tations of  hereditary  syphilis,  and  is  insichous  in  its  development,  with- 
out causing  functional  disturbances. 

Hereditary  sypMlitic  disease  of  the  fingers  after  the  first  year  of 
life  no  longer  shows  the  above  characteristic  and  unvarying  type;  caries 
may  now  appear,  as  well  as  involvement  of  the  joints  and  soft  parts. 

From  a  differential  diagnostic  standpoint  spina  ventosa  scrofulosa 
needs  consideration,  especially  if  only  the  basal  [jhalanx  of  one  finger  is 
affected.  Here  there  must  be  considered  the  history,  the  age  of  the 
child,  the  possible  presence  of  other  symptoms  of  syphilis,  especially  the 
characteristic  nasal  affection,  then  various  anatomical  factors  such  as 
absence  of  suppuration,  caries,  and  necrosis,  non-involvement  of  the 
skin  as  well  as  the  shape  of  the  diseased"  finger,  knob-shaped  in  the 
scrofulous,  olive  shaped  or  conical  in  the  specific  disease.  Appearance 
in  earhest  infancy  of  the  involvement  of  the  phalanges,  or  of  the  basal 
phalanges  of  all  fingers,  or  of  several  fingers,  would  always  speak 
for  syphilis. 

From  a  clinical  standpoint,  in  this  early  period,  only  those  bone 
changes  are  recognizable  in  which  the  periosteum  is  also  involved.  The 
finer  changes  at  the  cartilaginous  borders  of  the  bones  cannot  be  made 
out  by  palpation,  but  can  be  demonstrated  radioscopically. 


SYPHILIS 


529 


Very  frequently  a  radioscopic  examination  in  syphilitic  infants 
will  show  that  the  whole  osseous  system  has  been  changed  in  toto  while 
only  a  few  bones  will  show  any  pathological  changes  clinically.  (See 
Figs.  127,  128  and  129.) 

The  most  prominent  manifestations  are  swelling  in  the  region  of  the 
epiphyses  of  the  long  hollow  bones,  and  motor  disturljances. 

These  swellings  that  form  a  spindle-shaped  extension  from  the 
diaphysis  to  the  epiphysis  affect  especially  the  periosteum  and  the 
surrounding  soft  parts.  The  periosteum  appears  tliickened,  with  a  jelly- 
like infiltration  and  is  permeated  in  different  layers  by  ossifying  or 
chondrifying  proUferations.     Quite  frequently  all  of  the  tendon  inser- 

FiG.  131. 


Pseudoparalysis  of  the  right  upper  extremity  in  a  yypliililic  child  with  eruption.    Swelling  of  the  region  of  the 
elbow-joint.    .Spasm  of  the  hand.    Myotonia  of  the  extremities  that  are  free  from  paralysis. 


tions  and  all  of  the  muscle  bellies  surrounding  the  diaphyses  are  fused 
together  into  one  uniform  jelly-Uke  mass. 

Such  swelUngs  may  be  found  in  the  region  of  one  or  more  joints. 
The  elbow-joint  is  most  frequently  involved.  The  swollen  portion  is 
nearly  always  decidedly  tender. 

Two  kinds  of  motor  disturbances  must  be  distinguished,  the  para- 
lytic and  the  spastic.  The  latter  can  be  grouped  together  as  the  syphi- 
litic myotonia  of  infancy.  Myotonia  may  cause  inability  to  move  an 
extremity,  liut  is  nevertheless  always  a  spastic  comlition  as  ojjposed  to 
the  so-called  Parrot's  pseudoparalysis,  which  represents  a  flaccid  para- 
lysis of  the  arm  with  more  or  less  pain,  and  resulting  from  syjihilitic 
inflammatory  involvement  of  the  bone  and  muscles.  It  is  an  important 
fact  that  in  sypliilitic  bone  disease  of  the  lower  extremities  contractures 
result,  while  in  the  upper  extremities  we  have  instead  a  flaccid  paralysis, 
a  phenomenon  that   is  dependent   upon   the   different   relations  of  the 

II— .S4 


530  THE   DISEASES   OF   CHILDREN 

musculature  to  the  large  joints  in  the  upper  and  lower  extremities. 
The  occurrence  of  muscle  involvement  in  the  extremities  in  early 
hereditary  syphihs  is  of  great  importance  (Fig.  130). 

One  always  finds  specific  vascular  changes  in  the  affected  muscles 
in  which  interstitial  as  well  as  parenchymatous  and  degenerative  changes 
are  demonstrable.  The  nerve-fibres,  on  the  other  hand,  have  always 
remained  unaffected  in  those  cases  that  I  have  examined.  It  is  evident 
that  such  myopatliies  can  lead  to  paralytic  manifestations.  As  a  rule 
myostitis  starts  from  an  inflamed  periosteum,  but  is  may  arise  inde- 
pendently. A  warning  must  be  given  however  against  the  too  rapid 
diagnosis  of  gumma  in  the  case  of  a  nodule  in  the  sternocleidomastoid 
muscle  of  the  newborn  child.  Even  in  syphiUtic  cliildren  this  is  always 
a  traumatic  hematoma. 

The  clinical  manifestations  in  the  bone  system  in  early  heredi- 
tary syphilis  consist  of  swelling  of  the  bones  and  restricted  motion, 
which  are  often  associated  with  swelling  of  the  soft  parts,  separation  of 
the  epiphyses,  and  crepitation. 

The  possibiUty  of  the  occurrence  of  paralysis  of  central  origin  in 
early  hereditary  sypliihs  cannot  be  positively  denied.  It  must  be  said, 
however,  that  the  findings  that  we  have  up  to  the  present  time  (Sch- 
lichter,  Zappert,  von  Peters,  Scherer)  do  not  suffice  for  the  establishment 
of  a  spinal  basis  for  these  congenitally  syphihtic  motor  anomahes  in 
infancy.  In  most  cases  of  supposed  spinal  etiology  we  have  either  a 
simple  birth  palsy,  with  or  without  syphilis,  or  a  continuous  spasm  of 
toxic  origin,  wliich  may  occur  as  well  in  sypliihtic  as  jn  other  children. 
The  clinical  picture  of  this  form  of  paralysis  may  be  a  very  variable 
one.  Almost  without  exception  flaccid  paralyses  are  located  in  the  upper 
extremities  and  resemble  those  resulting  from  peripheral  plexus  lesions 
in  which  there  is  not  necessarily  always  much  pain  in  the  extremity. 
There  are  paralytic  manifestations  in  hereditary  sypliihs  in  which  the 
whole  upper  extremity  appears  paralyzed,  even  danghng;  then  again 
others  in  which  the  type  of  an  upper  arm  paralysis  is  most  prominent, 
and  finally  those  in  which  there  is  rather  a  forearm  type  of  paralysis. 

According  to  my  investigations  these  paralytic  manifestations  are 
always  the  result  of  muscle  involvement  alone,  which  probably  in  most 
cases  takes  its  origin  in  inflammatory  periosteal  affections.  Since  the 
muscle  disease,  in  the  absence  of  severe  periosteal  involvement,  is  not 
by  any  means  always  painful,  it  is  easily  seen  why  severe  pain  is  as 
frequently  absent  in  pseudoparalysis.  The  Ivlumpke-Dejerine  type  of 
paralysis  with  involvement  of  the  oculopupillary  fibres  is  not  a  form  of 
specific  motor  disturbance  in  infancy  but  is  rather  to  be  attributed  to 
obstetrical  plexus  paralysis,  if  found  in  hereditary  syphilitic  newborn 
and  older  infants. 

These  paralytic  appearances  may  manifest  themselves  suddenly, 


SYPHILIS  531 

in  one  night,  or  gradually.  In  tlic  upper  extremities  hereditary-syphi- 
litic bone  disea.ses  always  give  the  picture  of  flaccid  paralysis,  in  the 
lower  extremities  that  of  the  spastic  type. 

The  skull  in  hereditary  infantile  syphiUs  may  present  four  types 
of  changes: 

1.  Simple  rachitic  changes  which  manifest  themselves  in  softening 
of  the  bone  in  the  squamous  portions,  and  along  the  sutures,  and  are 
not  distinguishable  from  ordinary  racliitic  changes,  especially  from 
craniotabes.     These  frequently  occur  during  the  first  few  months  of  life. 

2.  Abnormal  protuberance  of  the  frontal  and  parietal  eminences 
with  striking  hardness  of  the  cranial  bones  and  of  the  sutures,  due  to 
an  early  periosteal  hyperostosis  of  these  bones.  Tliis  occurs  during 
the  first  few  months  of  hfe  but  is  not  so  frequent  in  early  infancy  as  the 
preceding  type. 

3.  Periosteal  sweUing  and  rarefaction  of  the  bone  in  isolated  or 
extensive  areas,  due  to  a  specific  inflammatory  involvement  of  the 
cranial  bones.  This  is  the  rarest  syphiUtic  lesion  of  the  skull  bones 
during  the  earliest  period  of  life,  and  is  characterized  by  areas  of  wast- 
ing (caries  sicca)  surrounded  by  walls  of  bone. 

4.  Hydrocephalus.  In  earliest  cliildhood  this  is,  in  a  good  many 
cases,  cau.sed  by  hereditary  syphilis.  The  latter  can  produce  inflam- 
matory changes  in  the  meninges  and  plexuses,  and  even  lead  to  disease 
of  the  intracranial  vessels,  simulating  tuberculous  meningitis.  Fre- 
quently, a  specific  diffuse  periostitis  of  the  inner  surface  of  the  cranial 
bones  is  the  starting  point  for  a  meningitis.  Specific  hydrocephalus  of 
infancy  has  a  superficial  resemblance  to  rachitic  pseudoh3^drocephalus, 
but  can  be  distinguished  from  it.  In  a  large  number  of  cases  syphi- 
litic hydrocephalus  is  curable  by  the  use  of  iodides  and  mercury. 

A  close  relationship  exists  between  hereditary  syphihs  and  rachitis. 

Among  children  afflicted  with  hereditary  sypliilis,  rachitis  is  some- 
what more  frequent  than  among  those  that  are  free  from  this  disease. 
Rickets  begins  earher  in  sypliilitic  children,  has  a  more  rapid  course,  but 
only  rarely  leads  to  a  high  degree  of  bone  deformity. 

The  circumference  of  the  skull  in  infants  with  hereditary  sypliilis 
is  greater  during  the  whole  of  the  first  year  than  in  those  that  are  nor- 
mal because  of  the  greater  bone  formation  at  the  centres  of  growth  of 
the  squamous  bones  due  to  specific  excitation.  During  the  first  half 
year  it  exceeds  that  of  rachitic  children,  and  only  during  the  second  half 
year  of  life  are  the  skulls  of  raclutic  children  larger  than  those  of  cliildren 
with  hereditary  syphilis.  As  a  consequence  there  is  a  peculiar  shape  to 
the  upper  surface  of  the  cranium,  characterized  by  the  prominence  of 
the  frontal  and  parietal  eminences,  while  between  the  latter  there  is  a 
more  or  less  well  marked  furrow.  This  cranial  anomaly,  called  caput 
natiforme  by  Parrot,  can  only  then,  with  certainty,  be  laid  to  heredi- 


532  THE    DISEASES   OF   CHILDREN 

tary  syphilis,  when  it  is  well  developed  during  the  first  few  months  of 
Ufa  and  is  associated  with  an  abnormal  hardness  of  the  skull  bones  and 
with  a  relatively  small  fontanelle;  the  same  shape  occurs  also  in  the 
heads  of  rachitic  children  who  are  free  from  sypliihs,  during  the  second 
and  third  year  of  hfe. 

If  the  caput  natiforme  is  associated  with  microcephalus  without 
well  marked  rachitic  changes  in  the  thorax  and  extremities,  this  speaks 
absolutely  for  hereditary  syphihs.  On  the  other  hand,  the  combina- 
tion of  a  high  degree  of  rachitic  deformity  of  the  extremities  and  this 
form  of  head  almost  positively  excludes  a  syphihtic  origin,  because  we 
know  from  experience  that  syphihtic  cliildren  only  exceptionally  be- 
come severely  rachitic. 

Liver  Affections. — Hereditary  syphihtic  disease  of  the  hver  in 
infancy  (.liffers  but  Uttle  anatomically  from  the  changes  previously 
discussed  that  occur  in  the  fcetus  and  the  newborn  child,  except  that 
syphilomatous  nodules,  considerable  induration  and  constriction  of  large 
portions  of  parenchyma  by  contracted  interstitial  cell  infiltration, 
together  v.ith  diffuse  cell  prohferation,  are  all  met  more  frequently. 

The  manifestations  in  the  hver  may  be  either  a  fresh  diffuse 
interstitial  process  wliich  causes  the  organ  to  increase  in  size  while 
remaining  soft,  or  an  indurative  inflammation  with  connective  tissue 
proliferation  and  palpable  increase  in  inconsistency.  A  termination  in 
cirrhotic  contraction  is  not  found  in  infancy.  Like\\-ise  I  could  never 
demonstrate  any  considerable  degree  of  ascites  in  hereditary  syphilis  of 
the  Uver  in  infancy,  while  in  later  childhood  icterus  as  well  as  ascites 
are  common  in  tliis  condition. 

The  most  important  chnical  symptom  is  increase  in  size.  I  found 
this  in  31  per  cent,  of  hereditary-sypliilitic  infants,  always  in  conjunc- 
tion with  a  var}ang  degree  of  enlargement  of  the  spleen.  It  must  be 
admitted  that  enlargement  of  the  hver  can  arise  also  from  stasis,  fatty 
infiltration,  etc.,  and  that  the  hver  can  normally  extend  more  than  1 
cm.  beyond  the  costal  border  in  the  parasternal  and  mammillary  hnes. 
A  comparison  however  of  the  frequency  of  occurrence  in  nonsyphihtic 
and  sypliiUtic  children  showed  that  in  scarcely  3  per  cent,  of  children 
under  six  months  old  in  whom  there  was  no  suspicion  of  syphihs  was  there 
present  a  projection  of  the  hver  beyond  the  costal  border  in  the  mam- 
millary hne  while  it  was  present  in  31  per  cent,  of  syphihtic  children. 
This  alone  would  seem  sufficient  to  prove  that  such  enlargement  of  the 
Uver  is  due  to  syphihs  even  if  there  is  no  demonstrable  hardening  of  the 
organ  present. 

Besides  this  simple  enlargement  of  the  hver  in  hereditary-syphihtic 
infants,  which  promj)tly  retreats  under  specific  treatment,  .there  is  still 
another  clinical  form,  the  hyperplastic  indurative  type,  in  which  the  organ 
in  some  cases  occupies  a  large  part  of  the  abdominal  ca%'1ty  and  can  be 


SYPHILIS  533 

felt  as  a  hard  body,  frequently  with  an  uneven  surface.  Children  af- 
fected with  tliis  form  of  liver  lesion  are  usually  born  with  it  in  its  fully 
developed  state;  they  show  a  distended  abdomen  with  well  marked, 
visible,  engorged  veins,  a  hard  enlarged  spleen,  and  rarely  also  icterus. 
The  length  of  life  of  infants  affected  with  tliis  form  of  liver  disease  is  a 
short  one.  Whether  early  hereditary  sypliilis  is  capable  of  producing  a 
hypertropliic  biliary  cirrhosis  has  not  been  decided  with  certainty.  Fre- 
quently congenital  icterus  due  to  obHteration  and  agenesis  of  the  gall- 
passages  has  been  attributed  to  syphilis,  but  erroneously,  unless  there 
were  other  manifestations  of  that  disease. 

Chiari,  Beck  and  others  have  described  in  the  newborn  a  gummatous 
inflammation  of  the  large  bile-passages  with  termination  in  contraction, 
induration  and  bile  stasis;  Schiippel  has  pointed  out  the  occurrence  of 
a  contracting  peripyophlebitis  in  syphiUtic  foetuses. 

It  is  an  important  fact  that  all  of  these  enlargements  of  the  hver 
accompanied  by  icterus  thought  to  be  due  to  syphiUs  were  present  at 
birth;  while  those  cases  that  occur  in  hereditary-sypliihtic  infants  after 
birth  run  their  course  as  a  rule  without  icterus. 

Affections  of  the  Kidneys. — It  is  certain  that  in  infants  ■with 
hereditary  syphihs  there  is  seen  during  the  stage  of  eruption  a  clinical 
picture  analogous  to  that  of  acute  nephritis,  which  disappears  under 
specific  treatment.  I,  myself,  then  Bradley,  Oedmansson,  and  Finkel- 
stein  have  seen  cases  of  that  sort.  On  the  other  hand  albuminuria  and 
casts  in  congenital  syphilis  are  frequently  due  to  severe,  compli- 
cating intestinal  disturbances,  without  having  any  connection  with 
syphiUtic  kidney  involvement,  the  existience  of  wliich  however,  as 
previously  stated,  is  undoubted  (Hecker,  Hochsinger,  Schlossmann, 
Karvonen,  Stroebe,  Stoerk). 

From  an  anatomical  standpoint  there  are  found,  besides  the  diffuse 
interstitial  cell  proliferation  of  the  connective  tissue  about  the  vessels, 
parenchymatous  changes  in  the  renal  epithelium  and  developmental 
anomalies  in  the  glomeruli  of  the  corte.x. 

CUnical  manifestations  on  the  part  of  the  circulatory  system  are 
rare  as  a  result  of  hereditary  sj'philis  in  infancy,  although  parenchy- 
matous, fibrous,  and  focal  lesions  of  the  heart  muscle  and  endocardium 
are  observed. 

The  relations  of  hereditary  syphilis  to  cardiac  and  vascular  changes 
will  be  discussed  more  fully  in  the  part  dealing  with  diseases  of  the 
circulatory  apparatus.  I  will  merely  mention  here  that  the  myocardium 
of  hereditary-sypliihtic  infants  is  very  frequently  occupied  by  foci  of 
coagulation  necrosis  which  were  once  erroneou.'^ly  thought  to  be  gum- 
mata,  and  that,  according  to  Winogradow,  there  are  changes  in  the 
automatic  gangUa  of  the  heart. 

Haemorrhages    from    the    navel    in    congenitally    syphilitic    new- 


534  THE   DISEASES   OF   CHILDREN 

born  infants  are  much  more  frequently  due  to  changes  in  the  walls  of 
the  umbilicial  vessels  which  hinder  their  contraction,  than  to  septic 
infection. 

•True  syphilitic  endoarteritis  is  usually  not  fully  developed  until 
the  second  half  of  the  period  of  infancy  and  then  involves  primarily 
the  cranial  vessels,  giving  rise  to  foci  of  cncephalomalacia.  General 
involvement  of  the  middle  arteries  has  often  been  observed  (Bergliinz). 

Children  \\ith  hereditary  sypliihs  frequently  have  distended  ves- 
sels of  the  skull  (Fig.  132)  wliich  are  not,  however,  as  E.  Fournier  thinks, 
due  to  a  parasypliilitic  dystrophy  of  these  veins,  but  are  caused  by 
hydrocephaUc  involvement  of  the  inside  of  the  skull.  In  the  same  man- 
ner the  medusa-like  appearance  of  the  veins  of  the  skin  of  the  trunk 
may  be  dependent  on  cirrhotic  changes  in  the  liver. 

Hereditary  sypliilis  of  the  lungs  occurs  occasionally,  though  rareh', 
in  infants  that  survive,  as  a  residue  from  the  diseased  fcetal  condition. 
As  a  rule,  lung  infiltrations  of  syphilitic  infants  depend  upon  second- 
ary infections. 

Early  hereditary  syphilitic  involvement  of  the  gastro-intestinal 
mucous  membrane  has  been  mentioned  before,  but  is  not  recognizable 
clinically.  Exceptionally  it  may  lead  to  haemorrhage  from  the  bowel 
as  a  result  of  ulceration  of  annular  infiltrations  of  the  mucosa. 

Changes  in  the  Central  Nervous  System. — These  are  not  rare 
during  the  eruptive  period  of  infantile  syphiUs  and  manifest  themselves 
clinically  by  great  restlessness  and  broken  sleep.  Increased  tension  in 
the  fontanelles  is  very  frequently  demonstrable  during  tliis  period  in  con- 
nection with  these  symptoms.  Involvement  of  the  brain  and  its  men- 
inges is  more  frequent  than  that  of  the  cord.  The  most  frequent  lesion 
to  be  found  in  this  connection  is  a  meningitis  serosa  interna  and  externa 
with  involvement  of  the  arachnoid  and  of  the  chorioid  plexus,  appearing 
in  the  form  of  an  acute,  or  chronic,  hydrocephalus.  This  very  frequently 
develops  during  or  at  the  end  of  the  first  eruptive  period,  or  occa- 
sionally in  connection  with  a  relapse.    More  rarely  it  is  present  at  birth. 

The  hydrocephaUc  head  of  congenital  syphilitic  origin  does  not,  as 
a  rule,  attain  the  enormous  size  of  the  nonspecific  form;  but  at  times, 
baloon-.shaped  enlargements  occur  in  that  form  of  syphiUtic  hydro- 
cephalus which  is  present  at  birth  (Figs.  132  and  133).  In  the  cases  that 
begin  after  birth,  an  early  hyperostosis  of  the  cranium  prevents  enor- 
mous enlargement.  Here  the  diagnosis  of  an  increased  amount  of  intra- 
cranial fluid,  a  miniature  hydrocephalus,  as  it  were,  must  be  made  from 
the  tense  and  bulging  fontanelle,  the  moderate  enlargement  of  the  head, 
and  the  characteristic  hydrocephaUc  facies.  This  syphiUtic  hydroceph- 
alus of  infancy  may  arise  very  acutely  with  the  picture  of  meningitis, 
or  it  may  occur  in.sidiously  without  marked  functional  disturbance  of 
the   central   nervous   system.      Finkelstein   has   stated    correctly   that 


SYPHILIS 


535 


Fig.  132. 


eclamp.sia  and  other  severe  nervou.s  symptoms  that  occur  during  the 
eruptive  days  of  hereditary  syphihs,  may  be  due  to  a  temporary  out- 
pouring of  hydrocephahc  fluid.  In  favor  of  this  view  are,  the  increased 
tension  in  the  fontanelle  that  is  out  of  all  harmony  with  the  miserable 
condition  of  the  child,  and  the  increase  of  pressure  as  shown  by  lumbar 
puncture.    The  latter  reveals  a  perfectly  dear  cerebrospinal  fluid. 

Besides  this  internal  serous  meningitis  there  occurs  in  infants  with 
hereditary  syphilis  a  pachymeningitis  hamorrhagica.  There  are  further 
inflammatory  changes  in  the  brain  and  meninges,  diffuse  and  circum- 
scribed, and  specific  vascular  lesions  with  resultant  tissue  changes,  that 
cannot  be  discussed  at  length  in  this  connection.  This  fact,  however, 
deserves  mention,  that  hydrocephalic  enlargements  can  result  in  heredi- 
tary syphilis,  from  these  condi- 
tions. If  hydrocephalus  is 
accompamed,  for  a  long  time,  by 
manifestations  of  paralysis,  or 
of  contractures,  then,  in  all  prob- 
ability, there  is  underlining  it  a 
complicating  brain  lesion. 

The  experience  that  a  great 
number  of  cases  of  hydrocephalus 
in  childhood  are  due  to  syphilis, 
as  a  result  of  true  or  parasypliil- 
itic  changes,  makes  it  a  duty  to 
use  antisypliilitic  treatment  in 
every  case.  As  a  matter  of  fact  it 
is  possible,  in  a  great  many  cases, 
to  cure  recent  cases  of  hydro- 
cephalus by  this  treat  men  t ,  and  to 
prevent  others  from  developing. 

Neuritis  involving  the  pe- 
ripheral  nerves  does  not  seem  to  occur   in   early  hereditary  syphilis. 

Clinical  manifestations  of  involvement  of  the  spinal  cord  in  early 
hereditary  syphilis  have  not  been  observed  hitherto;  and  yet  anatom- 
ical findings  are  admitted  by  Gilles  de  la  Tourette  and  Gasne,  who  found 
diseased  ves.sel  walls  and  interstitial  cell  proliferations,  as  well  as  dilTuse 
involvement  of  the  spinal  meninges,  in  both  newborn  and  older  infants 
with  hereditary  syphihs.  The  observations  by  Peters  of  specific  spinal 
paralysis  are  more  than  doubtful.  The  findings  of  Sibelius,  on  the  other 
hand,  deserve  consideration:  he  found  developmental  disturbancs  in 
the  cells  of  the  spinal  ganghon  in  newborn  sypliihtic  children. 

Ocular  Affections. — Syphilitic  affections  of  the  eyes  are  observed 
even  in  the  newborn  in  the  form  of  a  plastic  iritis  which  may  run  its 
course  within  the  uterus  and  mav  lead  to  svnecliia  of  the  iris.    Iritis  of 


Syphilitic  hydroceplialu5  with  a  high  degree  of 
engorgement  of  the  veins  of  the  scalp  and  with  a  well 
marked  nasal  deformity  (pug-nose). 


536 


THE   DISEASES   OF   CHILDREN 


early  hereditary  syphilis  runs  its  course  Avithout  the  \iolent  inflamma- 
tory manifestations  that  ordinarily  go  \Yith  this  affection.  A  diffuse 
optic  neuritis  may  occur  during  the  first  few  months;  still  more  fre- 
quently however  a  chorioiditis  with  the  formation  of  pecuHar  spots.  On 
the  other  hand  the  parenchymatous  keratitis  of  late  hereditary  syphihs 
is  very  rare  in  the  early  form.  It  must  be  remembered  further  that  in 
the  newborn,  traumatic  changes  brought  about  during  birth,  especially 
inflammatory  jirocesses  of  the  uveal  tract,  may  occur,  wliich  maj'  easily 
be  confused  with  syphilitic  lesions. 

The  organ  of  hearing  seems  to  be  involved  only  exceptionally  in  a 
specific  manner  in  infantile  syphilis,  although  a  discharge  from  the  ear 
does  occur  in  some  of  these  cases. 

Affections  of  the  Lymph-nodes. — The  torpid  polyadenitis  charac- 
teristic of  acquired  sypMhs  has  its  analogy  in  early  hereditary  syphilis 

Fig.  133. 


Sj-philitic  hydrocephalus.     A  high  degree  of  atropliy.     Saddle-nose  with  the  end 
of  the  nose  retracted.     Borders  of  the  lips  infiltrated. 


in  the  frequent  but  by  no  means  constant  appearance  of  general  glan- 
dular enlargement  during  or  soon  after  the  period  of  eruption.  If  one 
examines  carefully  the  inguinal  and  axillary  regions  of  somewhat  older 
infants  with  hereditary  syphilis  it  is  not  difficult  to  make  out  a 
definite  shght  glandular  enlargement.  It  is  a  much  more  impor- 
tant fact,  however,  that  glandular  bodies  appear  in  infants  with  early 
congenital  syphiUs  in  places  in  which  normally  no  glands  are  palpable, 
as  in  the  cubital  region,  and  in  the  fourth  or  fifth  intercostal  space  at 
the  side  of  the  thorax.  Regional  glandular  enlargement  that  is  not  de- 
pendent upon  ulcerated  skin  lesions,  but  in  extremities  that  are  the  seat 
of  bone  involvement  may  also  occur.  In  all  of  these  conditions  of  glan- 
dular enlargement  we  have  only  a-very  moderate  enlargement;  rarely  do 
these  lymph-nodes  become  larger  than  a  bean.  In  the  glandular  en- 
largement of  the  relapses  of  the  second  to  the  fourth  year  when  they 
are  associated  with  condylomatous  efflorescences  there  is  a  very  diff- 


SYPHILIS 


537 


erent  condition.     Here  we  have,  at  times,  lymph-nodes  that  attain  the 
size  of  hazel-nuts  with  no  tendency  to  suppuration. 

Complicating   Diseases   During   Infancy. — Congenitally    syphilitic 


™    3 


infants  have  a  very  marked  predisposition  to  septic  infection.  The 
cause  of  tliis  hes  in  the  many  chances  for  infection  due  to  the  purulent 
rhinitis  and  the  rhagadic  ulcerations  at  the  various  openings  into  the 
body.    It  is  not  necessary,  in  explaining  this  tendency,  to  resort  to  the 


538  THE   DISEASES   OF   CHILDREN 

idea  of  physical  inferiority  and  lack  of  resistance  of  the  tissues  brought 
about  by  congenital  syphilis,  when  the  avenues  for  infection  are  so  ex- 
traordinarily numerous.  It  is  not  difficult  to  explain  the  frequent  lung 
affections  of  the  syphiUtic  newborn  as  affections  due  to  a  purulent  nasal 
secretion  that  runs  downward,  or  the  frequent  skin  and  periarticular 
suppuration  as  due  to  metastases  from  various  primary  foci  of  suppuration. 

A  frequent  complication  is  found  in  pseudofiirunculosis  to  wliich 
especial  attention  must  be  drawn  because  of  the  fact  that  the  flaccid 
abscesses  that  occur  in  this  condition  are  frequently  considered  gum- 
mata.  Besides  these  exogenic  infections  of  the  subcutaneous  tissue 
there  occur  also  in  the  skin  of  congenitally  syphihtic  children  true 
cutaneous  furuncular  infiltrates  and  pustules  as  a  part  of  a  severe  gen- 
eral pya?mia.  Frequently  this  septic  secondary  infection  in  sypliiUtic 
children  manifests  itself  as  a  ha?morrhagic  disease  of  the  newborn,  in 
which  there  occur  niehpna,  purpura  and  bleeding  from  the  umbiUcus. 
For  a  long  time  a  specific  vascular  lesion  was  erroneously  thought  to  be 
the  cause  of  this  phenomenon  and  a  special  form  of  syphilis  hereditaria 
hcemorrhagica  was  thought  to  exist. 

When  occasionally  the  eruptive  lesions  of  early  hereditary  syphihs 
show  a  hsemorrhagic  character  this  can  doubtless  be  attributed  to  changes 
in  the  vessel  walls,  but  these  do  not  explain  fa'tal  internal  and  exter- 
nal haemorrhages  accompanied  by  general  dissolution  of  the  blood.  In 
these  cases  there  is  always  a  secondary  septic  infection  which  can  pro- 
duce hsemorrhagic  disease  in  the  syphilitic  newborn  child  as  well  as 
in  the  healthy  one.  Hess  distinguishes  three  forms  of  disease  in  this 
so-called  hemorrhagic  syphihs  of  the  newborn:  (1)  hsemorrhagic 
disease  without  evidence  of  syphihs,  but  with  sypliihtic  ancestors — an 
exclusively  septic  infection;  (2)  the  same,  but  in  children  who  are 
manifestly  syphihtic;  (3)  severe,  evident,  congenital  syphihs  with 
severe  internal  and  external  hsemorrhages. 

A  very  frequent  compUcation  of  early  hereditary  syphihs  is  found 
in  purulent  synovitis  of  the  larger  joints  which  is  not  infrequently 
associated  with  periarticular  abscess  formation. 

Parasyphilitic  Affections. — According  to  A.  Fournier  we  have  here 
tissue  changes  whose  final  cause  is  to  be  found  in  a  sypliihtic  infection 
but  without  the  real  products  of  syphihs,  i.e.,  real  syphihtic  lesions. 

These  affections  are  uninfluenced  by  specific  treatment  with  either 
mercury  or  iodides.  They  are  divided  into  organic  affections  that  are 
well  characterized  anatomically,  and  functional  disturbances  of  widely 
different  kinds  without  any  demonstrable  anatomical  ground. 

Hallopeau  has  recently  proposed  the  term  "syphihtic  deuteropa- 
thies"  for  these  changes.  Tommasoh  and  La  Mensa  designate  as"syph- 
ihsmus"  the  constitutional  disturbances  in  the  offspring  caused  by  the 
depraved  generative  cells  of  syphihtic  individuals. 


SYPHILIS  539 

If  individuals  who  present  genuine  manifestations  of  syphilis  in 
the  earliest  period  of  life,  show  evidence  of  parasyphilitic  diseases  later 
in  life,  this  is  not  hard  to  understand  and  is  not  to  be  distinguished  from 
the  corresponding  conditions  present  in  acquired  syphilis.  And  yet 
many  authors  consider  that  parasyphilitic  sj^mptoms  may  appear  in 
infancy  and  childhood  as  the  sole  manifestation  of  syphiHtic  influence 
upon  the  offspring  from  an  infected  ancestor,  without  such  children 
ha\ing  ever  shown  genuine  virulent  manifestations  of  syphilis.  This 
influence  they  tliink  may  extend  even  to  the  third  and  fourth  genera- 
tions (grandchild  and  greatgrandchild). 

Among  the  parasyphilitic  manifestations  the  following  are  the  most 
important:  ana?mic  and  atrophic  conditions,  failure  to  gain  in  weight 
and  to  develop  properly,  as  well  as  fcrtal  cachexia.  A  depraved  condi- 
tion of  the  generative  cells  of  the  parent  due  to  the  action  of  the  syphi- 
litic toxin  is  held  responsible  for  these  conditions,  much  as  in  the  off- 
spring of  a  tainted  parent  we  see  the  effects  of  alcohoUsm,  tuberculosis, 
saturnism,  mercurialism,  nicotism,  etc.  And  yet  there  is  little  real 
evidence  in  favor  of  such  an  influence,  since  one  can  always  assume  or- 
ganic changes  in  any  part  of  the  body  during  fcctal  hfe  and  one  can  think 
of  these  dystrophic  conditions  as  resulting  from  them.  Finger's  im- 
portant hypothesis  that  makes  a  distinction  between  purely  toxicogenic 
and  bacteriogenic  syphihtic  manifestations  explains  the  general  inferior- 
ity of  the  tissues  of  hereditary  syphiHtic  children  as  a  result  of  the  action 
of  toxins. 

A  further  division  into  meta-  and  parasyphilitic  affections  is  fre- 
quently made.  In  the  first  class  would  belong  anatomically  well  char- 
acterized organic  changes,  that  have  a  sypliilitic  base,  such  as,  especially, 
the  changes  in  the  central  nervous  system  (Mobius):  tabes,  dementia 
paralytica,  hydrocephalus,  cerebral  paralysis  of  cliildhood,  etc.;  to  which 
others  would  add  also  spastic  hemiplegia  and  diplegia.  In  the  second 
class  would  belong  the  general  disturbances  of  development  of  the 
heredosyphilitic,  among  which  one  would  consider  diabetes  mellitus 
and  insipidus,  as  well  as  the  hemoglobinuria  of  hereditary-syphilitic 
children. 

It  is  not  necessary  to  assume  a  harmful  parasyphilitic  protoplasmic 
change  to  explain  the  constitutional  disturbances  that  occur  during  the 
eruptive  period  of  early  hereditary  syphilis  and  that  are  characterized- 
by  pallor  and  stationary  weight.  Severely  affected,  atrophic,  syphilitic 
children  are  never  free  from  visceral  and  osseous  manifestations,  so  that 
the  cachexia  in  these  cases  can  be  directly  attributed  to  the  functional 
disturbance  of  organs  that  are  important  to  life.  The  irritation  of  the 
bone  marrow  which  is  never  absent,  and  which  is  characterized  by  the 
pouring  out  into  the  blood  of  characteristic  leucocytes  (myelocj'tic 
anaemia)  is  also  of  significance  in  this  connection.    It  has,  therefore,  by 


540  THE   DISEASES   OF   CHILDREN 

no  means  been  proven  that  the  high  mortality  of  hereditary-syphilitic 
infants  is  due  to  any  peculiar  tissue  inferiority.  Still,  the  influence  of 
fcrtal  syphihtic  inflammatory  processes  on  the  one  hand,  and  the  dis- 
turbances of  blood  formation  and  the  septic  secondary  infections  on 
the  other,  sufficiently  explain,  in  themselves,  the  high  mortality  of 
syphihtic  children. 

4.     SYPHILITIC   RELAPSES   IN  EARLIEST  CHILDHOOD 

Inasmuch  as  the  first  manifestations  of  syphilis,  in  a  great  many 
hereditary-sypliilitic  children,  run  their  course  within  the  uterus,  the 
whole  period  of  infantile  syphihs  and  the  first  eruption  are  a  relapse  in 
many  cases.  It  is  enough  to  mention  in  this  connection  the  ATSceral, 
osteochondritic  and  nasal  lesions  that  start  during  intra-uterine  life  and 
are  frequently  not  followed  hy  an  eruption  until  after  birth.  But  even 
after  the  first  eruption,  no  matter  whether  tliis  is  part  of  the  first  ap- 
pearance of  the  disease  or  not,  relapses  occur  during  infancy  in  the 
most  widely  different  organs.  Frecjuently  these  manifest  themselves 
as  a  continuous  progressive  development,  without  any  periods  of  la- 
tency, and  advance  until  a  large  number  of  organs  are  affected.  We 
find,  for  example,  that  certain  affections  of  the  bones  are  frequently 
associated  with  the  first  period  of  skin  eruption,  especially  those  of  the 
small  hollow  bones  (phalangitis)  and  the  periostitis  of  the  cranial  bones 
with  its  tendency  to  hyperostosis.  The  collection  of  hydrocephahc 
fluid  often  goes  hand  in  hand  with  these.  Not  infrequently  the  first 
exanthem  is  followed  by  an  unbroken  series  of  different  skin  lesions, 
such  as  recurring  infiltration  in  the  lower  half  of  the  body,  gumma 
formation,  oozing  condylomata,  and  mucous  patches.  Involvement  of 
the  organs  of  special  sense  hkewise  occurs.  In  general  the  following 
statement  is  true:  the  further  a  child  has  passed  beyond  the  period  of 
infancy,  the  less  do  cutaneous  lesions  manifest  themselves. 

Not  infrequently  congenital  syphihs  recurs  even  during  the  period 
of  infancy  in  the  form  of  affections  of  the  central  nervovs  system,  espe- 
cially as  cortical  encephaUtis  the  result  of  specific  vascular  lesions,  and 
as  hydrocephalus.  A  number  of  these  cases  run  their  course  as  a  cere- 
bral paralysis  of  childhood  with  or  without  epileptic  attacks,  and  are 
later  accompanied  by  disturbances  of  intelhgence.  It  is  an  important 
fact  that  sypliihtic  brain  cHseases  may  occur  in  infancy  \vithout  CAidence 
of  a  preceding  exanthem;  and  that  the  striking  result  of  the  use  of 
antisyphilitic  treatment  in  many  affections  of  the  brain  accompanied 
by  epileptic  seizures  in  earhest  childhood,  reveals  the  syphilitic  origin 
of  the  disease. 

Oc%dar  lesions,  either  independently,  or  in  specific  combination 
■with  other  sj'phihtic  lesions  due  to  relapses  are  much  more  frequent 
toward  the  end  of  the  period  of  infancy  than  during  the  first  period  of 


PLATE  27. 


m:M 


■UV. ■..,.■    . -^fec^^- 


,  -t^  "^    3        -r 


'•*  Si 


o    Si   S  _n    o    £ 


5  2  if  .". 


11  =  ".-= 
i  =  §-5:2 


a      -5-=  > 


—  ^ 
C  si 
3  "3 


rs     -^     S  e. 


,2  F-  e  «  - 


a  _    -   s  .-- 


o    »    -r  = 


2  • - 
=3   s 


.H   rt  rr   c   " 


;P    >.' 


S    c    g  ^ 

5  -^  =  o 


SYPHILIS  541 

eruption.  Above  all  others  in  importance  in  this  connection  is  the 
focal  choreorelinUis  of  Hirschherg,  which  is  most  characteristic  of  syph- 
ilis present  at  birth  but  which  also  appears  during  later  relapses.  Of 
less  significance  in  the  relapses  of  earhest  childhood  are  indurative 
lesions  of  the  palpebral  cartilages. 

Involvement  of  the  testicle  during  the  period  of  infancy  is  another 
form  of  relapse  in  congenital  sypliihs.  There  is  here  a  diffuse  inter- 
stitial cell  proliferation  analogous  to  the  chffuse  process  in  the  Hver 
(Hutinel).  This  manifests  itself  chnically  by  enlargement  and  indo- 
lent hardening  of  the  organ  (Henoch).  Since  this  lesion,  nearly  always 
bilateral,  is  accompanied  by  no  particular  subjective  symptoms  it  fre- 
quently escapes  notice  unless  it  is  looked  for  as  a  matter  of  routine  in 
the  cases  of  infantile  syphihs.  The  epididymis  always  remains  free. 
Syphilitic  orchitis  of  infancj'  never  leads  to  suppuration  and  is  a  pecu- 
liarly favorable  lesion  from  a  standpoint  of  therapeutics.  The  relapse 
manifestations  of  congenital  syphihs  during  infanc}'  are  by  no  means 
exhausted  by  those  organic  changes  that  have  been  enumerated  so  far. 
Especially  frequently  are  the  viscera  (Uver,  kidneys,  pancreas)  involved. 
The  thymus  also  may  be  affected,  at  times  becoming  very  large  and 
thus  producing,  as  Marfan  and  I  have  observed,  stenosis  of  the  trachea 
with  stridor  thymicus;  the  latter  disappearing  promptly  under  anti- 
syphiUtic  treatment.  Paroxysmal  htemoglobinuria  and  ha:'matoporphy- 
rinuria,  of  s^'pliihtic  origin,  may  occur  in  infancy. 

Quite  imperceptibly  at  times,  relapses  of  congenital  sypMlis  extend 
into  the  second  and  tliird  year  of  life,  locaUzing  themselves  especially 
in  the  skin,  in  the  ^^sible  mucous  membranes,  and  in  the  osseous  system. 

The  most  imjiortant  i<kin  lesion  of  hereditary  syphihs  of  the  second 
to  the  fourth  year  of  hfe  are  condylomata  lata  of  the  genito-anal  region; 
these  differ  in  no  way  from  those  of  acquired  sj^philis. 

The  visible  mucous  membranes  frequently  show,  even  up  to  the 
sixth  year  of  hfe,  sypliihtic  recurrences  in  the  form  of  mucous  patches, 
the  favorite  location  of  which  is  the  mucous  membrane  of  the  mouth  and 
pharynx  including  the  tonsils,  but  especially  the  upper  surface  of  the 
tongue.  On  the  latter,  extensive  manifold  condylomata  with  a  ten- 
dency to  repeated  recurrences  are  not  infrequent  (Plate  25). 

Recurrent  exanthemata  occur,  but  rarely,  up  to  the  middle  of  the 
second  year  of  life,  either  in  the  form  of  an  extensive  eruption  of  prom- 
inent papules  at  the  hairy  margin  of  the  forehead  or  on  the  flexor  sur- 
face of  the  extremities  (see  Plate  27),  or  in  the  form  of  inconspicuous, 
tender,  half-lentil  to  bean  sized  spots  on  the  forehead  and  the  lower 
part  of  the  body;  these  are  at  first  salmon  colored,  later  yellowish, 
shining,  and  somewhat  scaly.     The  trunk  is  nearl}-  always  entirely  free. 

Exanthemata  of  this  latter  variety  usually  occur  only  after  in- 
sufficient treatment  of  the  first  symptoms  of  sypliihs;   they  very  closely 


542 


THE   DISEASES   OF   CHILDREN 


resemble  the  eruptions  of  herpes  tonsurans  maculosus  and  are  fre- 
quently overlooked  or  misinterpreted.  Occasionally  there  are  found, 
even  during  the  second  year,  on  the  skin  of  the  extremities  and  of  the 
back,  small,  disc-shaped,  lightly  pigmented  spots,  not  raised  above  the 
surface,  wliich  take  on  a  bluish  violet  tint  and  become  clearly  marked 
when,  the  child  cries. 

Toward  the  end  of  the  first  and  during  the  second  year  there  fre- 
quently develops  the  cHnical  picture  of  pseudoleuksemic  anaemia  with 
marked  splenomegaly,  so  that  the  spleen  occupies,  as  a  hard  body,  the 
whole  left  half  of  the  abdomen  and  may  reach  down  into  the  pelvis. 
The  liver  is  always  considerably  enlarged  at  the  same  time.  In  many 
cases  of  hereditary  sypliilis  with  hepatic  and  splenic  enlargement  the 


Fig.  135. 


liver  is  much  larger  than  the  spleen. 
Here  there  is  a  real  syphihtic  involve- 
ment of  the  liver  with  an  indurative 
hyperplasia  of  the  spleen,  while  the 
type  first  described  is  a  manifestation 
of  pseudoleuksemic  antemia,  an  aflfection 
that  may  occur  without  preceding 
syphilis,  especially  in  severe  rickets. 

After  the  period  of  infancy,  con- 
genitally  syphilitic  disease  of  the  visceral 
organs  often  takes  on  a  true  gummatovis 
character  in  the  form  of  solitary  syphi- 
lomata,  which  may  occur  both  in  the 
parenchymatous  and  lymphatic  organs 
and  the  mucous  membranes.  A  nodular 
appearance  of  the  liver  is  very  frequent. 
Contracted  kidney  as  a  result  of  syph- 
iloma likewise  occurs  during  childhood. 
The  skin  and  subcutaneous  tissues 
occasionally  show  sohtary  gummata  in  the  years  following  infancy. 
These  appear  as  fluctuating,  dome-shaped  projections,  in  size  from 
that  of  a  bean  to  a  hazel-nut,  with  or  without  sHght  reddening,  and 
unaccompanied  bj^  fever.  If  recognized  early  and  treated  with  mercury 
these  rapidly  disappear.  If  not,  they  burst  and  empty  out  a  tough, 
sticky,  light  yellow  mass  and  are  converted  into  cup-shaped,  thick-walled 
ulcers  that  are  painless  and  without  reaction  and  heal  with  great 
difficulty  even  under  the  most  approved  treatment. 

A  rare  form  of  recurrence  is  to  be  found  in  a  diffuse,  sclerotic 
glo.ssitis,  which  von  Diiring  has  observed  repeatedly  in  endemic  sypliilis 
and  which  I  have  seen  three  times.  The  tongue  is  either  enlarged  in 
toto,  projecting  from  the  mouth,  much  as  in  myxccdema,  tlie  whole 
muscular  portion  being  uniformly  thickened  but  the  tongue  not  being 


Larynx  of  a  fifteen-months-old  child 
with  hereditary  .syphilis.  Growing  condy- 
lomata on  the  epiglottis  and  false  vocal 
chords.    Complete  aphonia. 


SYPHILIS  543 

cedematous  or  painful;  or  there  may  be  large  circumscribed  nodules  that 
occupy  from  one-third  to  three-fourths  of  the  whole  parenchyma  of 
the  tongue.  Such  indurations  of  the  tongue  occur  during  cliildhood 
only  with  sypliihs. 

Laryngeal  involvement  is  an  important  manifestation  of  recurrence 
of  hereditary  syphilis.  According  to  Ilipault  there  are  usuall}'  present 
nodular,  papular  growths  on  the  epiglottis  and  the  mucous  membrane 
of  the  vocal  cords,  that  break  down  into  ulcers,  and  later  form  scar 
tissue.  We  ourselves  have  seen  the  surface  of  the  vocal  cords  and  epi- 
glottis tliickly  covered  with  condylomata  in  a  child  of  15  months  with 
hereditary  syphilis  (see  Fig.  133). 

Laryngeal  syphilis  of  earliest  childhood  often  presents  the  picture  of 
croup  and  is  often  mistaken  for  croup;  in  some  cases  intubation  and 
tracheotomy  have  been  performed  to  prevent  suffocation.  Hereditary 
laryngeal  syphiUs  is  comparatively  rare  during  the  third  and  fourth 
years,  but  becomes  more  frequent  again  during  the  period  of  late 
syphilis.  The  essential  symptom  is  hoarseness,  even  complete  aphonia, 
without  a  demonstrable  catarrhal  condition  of  the  deeper  air-passages. 

5.     LATE   HEREDITARY   SYPHILIS 

By  late  hereditary  syphilis  we  mean  all  organic  affections  of  later 
childhood  and  of  adult  life,  that  are  caused  by  hereditary  syphihs  and 
that  are  analogous  to  the  teritary  manifestations  of  the  acquired  form. 
Age  is  of  less  significance  here  than  the  pecuhar  manifestations  of  syph- 
ilis; the  gumma  being  the  foundation  of  late  hereditary  syphihs.  As  a 
rule  gummatous  processes  in  hereditary  syphihs  do  not  arise  before  tlie 
fifteenth  year,  but  when  they  occur  earlier,  as  they  do  exceptionally, 
even  in  the  third  and  fourth  year,  they  must  be  classed  nevertheless  as 
manifestations  of  late  hereditary  syphihs.  (See  Recurrences  in  Early 
Childhood.) 

There  are  authors  who  still  beheve  in  the  possibility  of  a  latent 
period  in  hereditary  syphihs  to  the  time  of  puberty,  and  who  consider 
only  such  cases  as  late  hereditary  sj'pliihs  in  which  tertiary  symptoms 
have  not  been  preceded  by  early  manifestations  of  sypliihs  during  the 
first  few  years  of  hfe.  There  are  no  plausible  grounds  for  assuming  such 
an  outbreak  of  tertiary  symptoms  alone.  In  chiklren  who  apparently 
have  late  hereditary  syphihs  alone,  early  manifestations  may  have  been 
overlooked  because  of  the  possible  absence  of  eruptions,  or  there  may 
have  been  a  contact  syphilis  that  ran  its  course  early  in  childhood  and 
escaped  observation. 

As  the  extreme  age  at  which  condylomatous  recurrences  may  take 
place  in  hereditary  syphilis  I  would  designate  the  sixth  j^ear,  during 
which  year  I  have  still  found  condyloii,iata  of  the  nuicous  membrane. 
As  a  rule  these  do  not  occur  even  after  the  fourth  year. 


544  THE   DISEASES   OF   CHILDREN 

In  late  hereditary  syphilis,  genuine  sypluUtic  manifestations  are 
much  more  sharply  distinguished  from  parasypliilitic  affections  than  in 
the  early  period  of  the  disease,  these  parasypliihtic  manifestations  in- 
vohing  especially  the  central  nervous  system  in  the  form  of  tabes  and 
paralytic  dementia.  Late  hereditary  sypliiUs  frequently  appears  first 
during  the  period  of  second  dentition  or  during  pulierty. 

A\'e  will  now  discuss  briefly  in  the  order  of  their  frequency  the  lesions 
of  the  different  organs  involved  in  late  hereditary  syphilis. 

First  are  affections  of  the  eye  which  will  only  be  mentioned,  leav- 
ing their  detailed  discussion  to  the  ocuhst.  Here  belong  parenchymatous 
keratitis,  gummatous  involvement  of  the  iris  and  the  so-called  deep 
inflammations  of  the  eye,  choreoretinitis  and  optic  neuritis. 

Very  frequently  ocular  and  aural  lesions  appear  at  the  same  time, 
but  the  latter  usuall}'  occur  later.  "When  the  ears  are  affected,  there  is 
rapid,  usually  bilateral,  deafness.  It  occurs  much  more  frequently  after 
puberty  than  before  and  is  accompanied  by  symptoms  of  dizziness  and 
subjective  noises.  Deafness  is  due  to  neimtis  acvstica  (Meniere's 
disease). 

Late  syphilitic  changes  in  the  osseous  system  take  place,  either  as  a 
diffuse  hyperplastic  ostitis  and  periostitis,  or  as  a  gummatous  process; 
both  lesions,  however,  may  occur  not  only  simultaneously  in  the  same 
individual  but  also  in  the  same  bone. 

The  hyperplastic  ostitis  and  periostitis  may  involve  the  whole 
skeleton  (Lannelongue)  and  may  cause  visible  swelhngs  of  the  cranial 
bones  as  well  as  of  the  long  hollow  bones  (see  Plate  29).  And  yet  hyper- 
plastic processes  in  the  cranial  bones  occur  incomparably 'less  frequently 
in  late  than  in  early  herechtary  sypliihs.  The  long  hollow  bones  are 
most  frequently  affected,  especially  the  shinbones,  their  involvement 
in  late  hereditary  syphiUs  forming  an  extremely  characteristic  chnical 
picture.  In  general,  all  of  the  bone  lesions  of  tertiary  acquired  syphihs 
occur  also  in  late  hereditary  sj'pliihs.  Lannelongue  considers  the  so- 
called  Paget's  bone  disease,  which  is  a  cUffuse,  progressive  periostitis 
leading  to  hyperostosis  and  finally  affecting  the  whole  skeleton,  as  noth- 
ing more  than  hereditary  bone  sypliilis. 

As  mentioned  above,  the  tibia  is  the  bone  most  frequently  involved. 
There  occur  at  first  on  the  crest  of  the  tibia  rather  soft  and  very  tender 
swellings.  The  skin  over  these  areas  is  frequently  shghtly  reddened  and 
sensitive.  At  the  same  time  there  is  frequently  spontaneous  pain  in  the 
lower  extremities  increased  decidedly  on  attempting  to  move  them. 
The  first  stage  of  the  affection  during  which  new  tophi  appear  one 
after  the  other  on  the  edge  of  the  tibia,  may  last  for  months  or  may 
progress  so  rapidly  that  they  resemble  erythema  nodosum.  The  second 
stage  is  the  real  stage  of  hyperostosis.  The  entire  shin  becomes  swollen, 
thickened,  and  the  sharp  edge, of  the  bone  disappears.     The  anterior 


SYPHILIS  545 

border  of  the  bone  becomes  rounded  and  is  covered  with  palpal)le  hard 
nodules  and  rough  areas.  The  whole  bone  is  diseased.  Anatomically 
this  deformity  is  due  to  the  continuous  formation  of  new  periosteal 
bone  layers  about  the  primary  bone.  Even  the  hollow  portion  of  the 
bone  is,  in  many  cases,  obhterated  for  considerable  distances  and  is 
filled  bj'^  compact  bone  substance.  The  tibia  is  frequently  bent,  with 
marked  anterior  convexity,  the  bone  assuming  the  shape  of  a  Turkish 
sabre;  or,  if  the  o.ssifying  periostitis  of  the  anterior  margin  of  the  tibia 
far  exceeds  that  of  the  other  surfaces,  we  have  the  sabre  sheath  defor- 
mity of  Hutchinson  and  A.  Fournier  (see  Plate  29). 

Among  the  less  frequent  bone  changes  in  late  hereditary  sypliilis  is 
a  rarefying  periostitis  leading  to  bone  absorption.  This  occurs  occa- 
sionally on  the  surface  of  the  cranial  bones  and  may  lead  to  extensive 
resorption  of  bone  with  the  formation  of  rough  areas  on  the  surface 
(caries  sicca). 

Just  as  in  the  tertiary  stage  of  acquired  sypliilis,  there  occur  at 
times  in  late  hereditary  sypliiUs  nodular  periosteal  swellings  (gummata) 
with  their  favorite  location  again  in  the  tibia,  but  also  in  the  bones  of 
the  upper  extremit}',  the  cranium,  and  the  sternum.  These  are  at  first 
rather  soft,  sensitive  swelhngs  that  are  surrounded  by  a  wall  of  hyper- 
ostosis. They  either  heal,  leaving  a  depression,  or  discharge  and  become 
converted  into  thick  walled,  excavated  ulcers  which  heal  with  the 
formation  of  adherent  scars.  If  the  gummatous  process  is  centrally 
located,  bone  necrosis  may  result. 

The  short  hollow  bones,  too,  may  be  involved  in  late  hereditary 
syphilis,  but  these  lesions  no  longer  show  a  typical  course  with  pref- 
erence for  the  first  phalanges  of  the  fingers,  but  occur  without  any 
definite  rule  in  the  phalanges,  carpus,  and  bones  of  the  foot. 

The  lesions  of  the  nose  and  of  the  alveolar  processes  show  no  differ- 
ences in  acciuired  and  in  late  hereditary  syphihs.  Perforations  of  the  bone 
that  are  rare  in  early  syploiUs,  are  of  frequent  occurrence  at  this  stage. 

Not  infrequently,  hereditary  syphihs  leads  to  lengthening  of  the 
affected  long  bones  in  older  cliildren.  Tliis  is  especially  striking  in  the 
occasional  lengthening  of  a  lower  extremity,,  hke  a  partial  gigantism,  in 
wluch  the  affected  bones  become  plump  and  rougher.  Frequently  such 
bone  changes  are  preceded  for  months,  even  years,  by  severe  nocturnal 
headaches. 

In  late  hereditary  syphilis  genuine  syphilitic  joint  lesions  of  a  very 
definite  type  occur.  The  knee-joints  are  most  frequently  involved,  at 
times,  even  during  }-outh  and  adult  life.  According  to  my  experience 
two  main  types  can  dc  chstinguished: 

1.  Joint  affections  without  involvement  of  the  hone  and  cartilage. — 
These  naturally  fall  into  two  subdi^•isious:  (o)  simple  hydrops 
of   the    joints    without    material    tliickening    of     the    joint     capsules, 

11—35 


546  THE    DISEASES   OF   CHILDREN 

the  synovial  membranes  and  the  tendon  sheaths;  running  an  afebrile, 
almost  painless  course;  it  is  almost  exclusively  h  mi  ted  to  the  knee  and 
ankle-joints  and  causes  a  shght  feehng  of  weariness  and  weight  in  the 
legs;  (6)  hyperplastic  synovitis.  In  this  form  the  joint  capsule,  the 
synovial  membranes,  together  with  the  tendon  sheaths,  are  all  thick- 
ened and  almost  gelatinous.  Friction  rubs  can  usually  be  heard  and 
felt  in  these  joints.  Tliis  process  may  involve  the  phalangeal  and 
carpal  joints,  then  the  smaller  joints  of  the  foot,  in  fact  may  spread  to 
every  joint  in  the  body,  including  those  between  the  vertebrie,  as  a 
generahzed  hyperplastic  arthromeningitis. 

2.  Joint  affections  with  enlargement  of  the  ends  of  the  bones. — 
These  hkewise  fall  into  two  subdivisions:  (a)  combination  of  hydar- 
throsis  with  swelling  of  the  joint  ends  of  the  hollow  bones.  This 
is  the  most  common  form  of  joint  affection  in  late  hereditary  syphiUs, 
resembling  not  a  httle  the  chnical  picture  of  arthritis  deformans.  It  is 
important  from  a  diagnostic  standpoint  that  the  swollen  bone  ends  are 
not  sensitive  to  pressure.  The  Rontgen  ray,  too,  shows  there  no  peri- 
osteal layer  formation  as  in  the  afTections  of  the  diaphysis;  (b)  an  affec- 
tion simulating  white  sweUing.  This  is  usually  monarticular,  painful, 
and  is  associated  with  limitation  of  motion,  hke  the  tuberculous  form. 
It  may  lead  to  rupture  and  to  necrosis. 

It  is  especially  important  for  the  pediatrist  to  be  able  to  distinguish 
between  tuberculous,  or  scrofulous,  and  syphihtic  bone  and  joint  affec- 
tions. In  general,  caries  is  more  frequent  in  tuberculosis  than  in  heredi- 
tary syphihs.  The  syphihtic  bone  afTections  are  less  tender  than  those 
due  to  tuberculosis  and  produce  fewer  functional  disturbances.  In  bone 
syphihs  of  later  childhood,  too,  there  is  an  absence  of  hectic  fever  and 
of  the  profound  cachexia  of  bone  tulierculosis.  In  the  cases  that  go  on 
to  suppuration  the  nature  of  the  skin  involvement  may  lead  to  a  diff- 
erential diagnosis.  The  pecuhar  character  of  the  skin  in  scrofulous 
caries,  the  violet  discoloration,  the  manner  of  perforating,  the  formation 
of  multiple  fistulas,  and  the  spongy  granulations,  all  speak  unquah- 
fiedly  for  tuberculosis  and  are  absent  in  syphilis. 

In  case  of  bone  swelhng  without  suppuration  the  locaUzation  is  of 
some  importance.  In  general  the  cranial  bones  are  more  prone  to  syph- 
ihs than  to  tuberculosis,  and  especially  the  frontal  and  parietal  bones, 
particularly  their  eminences.  Only  the  temporal  and  malar  bones  are 
more  frequently  the  seat  of  tuberculosis  than  of  sypliihs.  Affections  of 
the  occipital  bone  again  are  as  a  rule  syphihtic  in  children. 

The  skin  lesions  of  late  hereditary  syphihs  differ  in  no  way  from  the 
tertiary  skin  lesions  of  acquired  syphilis.  According  to  my  experience 
there  are  two  main  forms  of  true  skin  lesions:  the  small  nodules,  and  the 
large  nodular  late  syphihdes.  In  the  former  they  are  circumscribed  in- 
filtrations of  the  skin  that  feel  hard  at  first  and  vary  in  size  from  a  spht 


SYPHILIS  547 

pea  to  a  lentil.  The  skin  over  these  nodules  becomes  brownish  in  color 
and  either  desquamates  or  I^eeonies  covered  with  a  crust.  These  nodules 
are  usually  grouped  together  closely,  very  nmch  as  in  lupus  vulgaris. 
Below  the  crusts  the  granulation  tissue  disintegrates,  while  the  crust 
itself  grows  larger  and  assumes  the  shape  of  a  cup,  or  of  an  oyster  shell. 
Some  nodules  may  undergo  resorption.  In  general  these  late  small 
nodular  syphilides  show  a  serpiginous  arrangement. 

This  serpiginous  syphilide  of  childhood  belongs  to  the  most  intract- 
able manifestations  of  syphilis,  from  a  therapeutic  standpoint. 

The  large  nodular  syphilide  occurs  in  the  form  of  large  skin  gum- 
mata,  and  gummatous  ulcers,  though  not  a  frequent  lesion.  The  point 
of  origin  is  usually  the  subcutaneous  tissue. 

The  mucous  membranes  also,  especially  those  of  the  respiratory 
tract  become  invaded  in  a  specific  manner  in  late  hereditary  syphilis. 
It  is  not  always  possible  to  determine  whether  the  gummatous  process, 
in  tliis  case,  starts  in  the  nuicous  membrane  itself  or  in  the  deeper  King 
tissues.  Tills  is  especially  true  of  the  lesions  of  the  nasal  and  pharyn- 
geal mucous  membranes.  In  older  children  the  differential  diagnosis 
between  ulcers  of  the  skin  and  mucous  membranes  due  to  tuberculous 
lupus  on  the  one  hand  and  those  due  to  syphilis  on  the  other  hand, 
are  to  be  considered.  Rapidly  progressive  ulceration  with  absence  of 
nodular  infiltration  always  speaks  for  syphilis.  Pharyngeal  and  laryn- 
geal ulcers  due  to  hereditary  sypliilis  are  characterized  by  sharply 
defined  borders  and  by  thick  walls. 

As  far  as  the  nose  is  concerned,  a  diffuse  osseous  and  periosteal 
affection  of  the  whole  nasal  skeleton,  or  the  formation  of  gummata 
within  the  cartilaginous  or  bony  nasal  septum  or  at  the  base  of  the  nasal 
cavity,  may  represent  the  primary  pathological  process;  breaking  down 
of  the  affected  tissues  maybe  followed  by  ulceration  of  the  mucous  mem- 
brane. And  yet  circumscribed  nodules  may  form  on  the  mucous  mem- 
brane of  the  cartilaginous  and  soft  portions  of  the  nose  and  these  maj^ 
lead  to  ulcer  formation.  Probably  the  most  frequent  lesion  is  a  gumma- 
tous ostitis  of  the  liony  portion  of  the  septum,  the  first  symptom  of  which 
is  an  obstinate  nasal  obstruction.  The  gummatous  ulceration  is  always 
accompanied  by  much  pus  and  crust  formation.  If  proper  therapeutic 
measures  are  not  instituted  at  the  right  time,  deep  ulceration  will  take 
place  with  perforation  of  the  septum,  or  the  floor  of  the  nose,  together 
with  necrosis  of  the  affected  portions  of  the  ethmoid  and  the  superior 
maxilla.  The  final  outcome  of  such  nasal  disturbances  will  be  dis- 
cussed in  connection  with  the  discussion  of  the  stigmata  of  hereditary 
syphilis. 

Atropine  rlunopharyngitis,  or  oza>na,  is  a  frequent  syphihtic 
deuteropathy  in  children  between  six  and  fifteen  years  of  age.  The 
smooth  atrophy  of  the  base  of  the  tongue  (Levin,  Heller)  characterized 


548  THE   DISEASES   OF   CHILDREN 

by  smoothness  and  thinness  of  tlie  mucous  membrane  and  b}'  absence 
of  glands,  likewise  occurs  as  a  luetic  deuteropathy  in  children  of  the 
al30ve-mentioned  age  with  hei'cditary  syphilis. 

The  palate  and  pharynx  become  invaded  very  frequently  and  in  a 
very  characteristic  manner  in  late  hereditary  syphilis.  I  would  men- 
tion first  the  syphihtic  tophus  of  the  hard  palate,  usually  projecting 
from  the  raphe,  a  lesion  which  contrasts  strongly  with  the  purely  mucous 
membrane  affections  of  herechtary  syphihs  on  account  of  its  painful 
character,  and  which  represents  a  stage  preceding  ulcerative  palate  per- 
foration. Furthermore,  gummata  occur  on  the  velum  palati  and  within 
the  palatine  arches,  and  may  lead  to  deep  ulceration  and  perforation. 
The  favorite  location  is  the  point  of  insertion  of  the  uvula  and  the 
middle  portion  of  the  anterior  palatine  arch,  where  at  first  painless 
swellings  arise.  These  reveal  their  presence  only  by  a  shght  pecuHarity 
in  the  voice  sounds  such  as  exists  with  a  tonsillar  abscess,  but  they  do 
not  materially  interfere  with  swallowing.  Not  until  ulceration  or 
perforation  has  taken  place  do  we  notice  the  well-known  functional 
disturbance  of  speech  and  swallowing. 

Circumscribed  or  diffuse  swelhngs  arise  also  on  the  mucous  mem- 
branes of  the  epiglottis  and  of  the  larynx  that  are  of  great  importance 
because  of  their  interference  with  speech  and  respiration.  The  late  syph- 
ilitic ulcers  of  the  nasopharynx,  larynx  and  trachea  have  a  decided  ten- 
dency to  the  formation  of  scar  tissue  with  contractions.  For  that  reason, 
very  characteristic  sequel£e  of  late  hereditary  sypliihs  are  such  lesions  as 
cicatricial  adhesions  of  the  velum  palati  to  the  posterior  pharyngeal 
wall,  contractions  and  distortions  of  the  epiglottis,  stenosis  of  the  larynx 
and  of  the  trachea,  the  latter  being  among  the  most  serious  lesions  from 
a  therapeutic  standpoint. 

Among  the  \isceral  lesions  of  late  herechtary  sypliihs,  so  far  as  fre- 
quency is  concerned,  hver  affections  deserve  the  first  rank,  although  the 
lesions  differ  in  no  way  from  those  occurring  during  tertiary  acquired 
sypliihs.  The  large  nodular  gumma,  the  diffuse  connective  tissue  hyper- 
tropliic  cirrhosis,  and  the  characteristic  lobulated  hver,  resulting  from  a 
combination  of  these  two  conditions,  deserve  mention.  These  hver 
changes  are  always  associated  with  hyperplasia  of  the  spleen. 

Many  cases  of  contracted  kidney  and  of  amyloid  degeneration  of 
the  kidneys  may  be  a  manifestation  of  late  hereditary  sypliihs.  It  is 
certain  also  that  diabetes  insipidus  in  infants  is  frequently  associated 
with  hereditary  sypliihs. 

Lesions  of  the  circulatory  apparatus  in  late  hereditary  sypliihs 
will  be  discussed  in  the  chapter  on  diseases  of  the  circulatory  system. 
It  should  be  mentioned,  however,  in  this  connection,  that  gummata 
aortitis,  as  well  as  arteriosclerosis  and  phlebosclerosis,  occurs  in  con- 
genitally  syphihtic  children,  and  that  the  presence  of  the  latter  condi- 


SYPHILIS  549 

tions  during  childhood  always  justifies  the  diagnosis  of  syphilis.  Myo- 
cardial and  endocardial  changes  are  observed  in  late  hereditary  sypliilis. 

Hyperplasia  of  the  adenoid  tissue  in  the  nasopharynx  is  a  very  fre- 
quent finding  in  hereditary  sypliihs,  without  liowever  having  any  pecu- 
liar characteristics  that  are  of  diagnostic  value.  Not  alone  is  Luschka's 
tonsil  hypertropliied,  but  also  the  remainder  of  the  adenoid  ring  of  the 
throat,  the  tonsils,  and  the  adenoid  tissue  at  the  base  of  the  tongue.  In 
the  latter  position  two  diametrically  opposite  conditions  can  exist :  a 
smooth  atrophy,  and  adenoid  hypertrophy. 

On  account  of  the  great  frequency  of  adenoid  vegetations  in  the 
nasopharynx  in  older  cliildren  -with  hereditar}'  syphiUs,  there  are  nearly 
always  present  enlarged  submaxillary  and  cervical  lymjih-nodes.  Natu- 
rally, these  must  not  be  looked  upon  as  a  specific  adenopathy,  although 
such  a  condition  can  exist  in  late  hereditary  syphilis.  There  is  a  certain 
tendency  to  glandular  enlargement  even  in  these  older  syphiUtic  chil- 
dren, so  that  Ivmph-nodes  can  he  felt  in  places  where  there  are  none 
palpable  normally,  as  for  example,  in  the  cubital  region  [epitrochlear 
lymph-nodes.] 

Besides  these  simple  lymph-node  hypertropliies,  there  occur 
though  not  frequently,  genuine  gummata  of  the  lymph-nodes.  This 
may  take  one  or  two  forms:  either  an  enlargement  of  a  single  node,  or 
group  of  nodes,  that  are  hard  and  painless  and  have  but  little  tendency 
to  softening;  or  as  a  generalized  lymph-node  hypertrophy,  very  sim- 
ilar to  the  polyadenitis  of  the  secondary  stage  of  acquired  syphihs, 
except  that  in  the  late  hereditary  sypliilis  of  childhood  the  nodes 
become  larger  than  in  the  other  condition. 

If  these  are  only  isolated  enlarged  lymph-nodes,  the  differential 
diagnosis  from  tuberculous  lymphadenitis  is  not  alwaj^s  easy.  As  a  rule 
stigmata  of  syphihs  are  present  in  the  one  case,  and  help  in  making  a 
diagnosis.  The  more  marked  appearance  of  periadenitis  in  the  syphihtic 
form  is  of  some  diagnostic  value.  The  course  of  suppuration  of  a  gum- 
matous node  is  wholly  different  from  that  of  a  tuberculous  node.  In  the 
breaking  down  of  a  gummatous  node  a  circumscribed  portion  of  the 
swelhng  unites  with  the  skin  and  the  latter  breaks  down  rapidly  over  a 
considerable  area,  gi\Tng  rise,  after  the  emptjing  of  the  characteristic 
contents  of  the  gumma,  to  an  ulcer  with  all  of  the  characteristics  of  the 
sypliilitic  type.  The  gummatous  involvement  is  exceedingly  amenable 
to  antisyphihtic  treatment,  a  fact  that  is  of  great  value  from  a  diagnostic 
standpoint. 

Hereditary  syphilis  frequently  causes  general  nervous  chsturbances, 
even  a  nervous  predisposition.  Many  of  these  children  are  feeble-minded. 
Difficulty  in  learning  and  in  obser\ing,  and  attacks  of  night  terrors  are 
frequently  attributed  to  adenoids  in  the  nasopharynx.  Init  operation  in 
these  cases  leads  to  no  improvement  and  shows  that  there  is  some  more 


o 


550  THE   DISEASES   OF   CHILDREN 

fundamental  cause.  Psychoses,  too,  occur  in  these  cliildren  at  the  time 
of  puberty  (Dornblueth).  To  tliis  period  belong  infantile  tabes  and 
progressive  paralysis,  while  epilepsy  of  the  Jacksonian  type  is  possible 
at  any  time  during  childhood,  suiicrimposed  upon  the  same  syphilitic 
foundation.  These  diseases,  together  with  brain  and  spinal  syphilis 
of  the  second  period  of  cliildhood,  and  the  pseudotabes  of  hereditary 
syphilis,  are  all  discussed  in  their  appropriate  place  in  that  part  of  tliis 
work  that  deals  with  diseases  of  the  nervous  system. 

Stigmata  of  Hereditary  Syphilis. — In  a  great  many  cases  heredi- 
tary syphilis  leaves  lasting  changes  by  which  it  can  be  recognized. 
These  are  especially  well  marked  during  the  second  period  of  cliildhood, 
wliile  some  of  them  may  disappear  during  later  life.  These  stigmata 
consist  of  general  disturbances  of  development,  of  cutaneous  scars,  of 
certain  skeletal  changes,  and  of  the  so-called  Hutcliinson's  triad. 

1.  General  disturbances  of  development  are  especially  significant  at 
the  time  of  puberty,  because  infantilism  is  frequently  observed  with 
dwarfism  and  retarded  sexual  development.  Tliis  infantilism  occurs  as  a 
secjuel  to  severe  early  hereditary  syphilis  from  which  the  child  recovered 
in  earliest  infancy  and  is  distinguished  from  dwarfism  due  to  other 
causes  by  the  invariable  presence  of  other  unmistakable  manifestations 
of  syphilis. 

2.  Scars. — The  ulcerative  skin  affections  of  hereditary  syphilis 
leave  scars,  the  location  and  configuration  of  wliich  frequently  make 
possible  a  retrospective  diagnosis  of  hereditary  sypliilis,  without  any 
knowledge  of  its  previous  manifestations.  The  radially  arranged  cir- 
cumoral,  circumnatal  and  circumanal  scars  and  the  scar  formations  on 
the  mucous  membranes  of  the  palate,  pharynx,  and  larynx  are  very 
significant.  The  most  important  scar  symptoms  are  the  radially 
arranged  cicatrices  on  the  lips  which  give  the  latter  a  generally  paler 
and  wrinkled  appearance. 

3.  Skeletal  Changes. — The  bone  changes  of  the  first  few  years, 
as  well  as  those  of  later  childhood,  leave  permanent  bone  deformities. 
Among  these  are  peculiar  deformities  of  which  Parrot's  natiform  caput 
has  been  mentioned  repeatedly.  There  is  present  a  thickening  of  the 
frontal  and  parietal  eminences  and  a  broadening  of  the  transverse  diam- 
eter of  the  skull,  so  that  there  is  formed  a  more  or  less  deep  furrow,  not 
unlike  the  intergluteal  fold.  Frequently,  too,  there  is  associated  a  prom- 
inent bulging  forward  of  the  whole  abnormally  high  broad  forehead, 
with  especially  marken  projecting  and  rounded  eminences,  the  so-called 
"Olympic  brow."  There  are  observed  also  cranial  asymmetries,  that  are 
not  however,  always  to  be  attributed  to  syphilis,  but  rather  to  a  cranial 
hyperostosis  of  .syphihtic  origin  combined  with  racliitic  changes.  Hydro- 
cephalic and  microcephalic  heads,  like^\^sc,  are  found  as  a  result  of  hered- 
itary syphihs.   Permanent  deformities  of  the  nasal  skeleton  are  frequent, 


PLATE  28. 


■  S 


la 


SYPHILIS  551 

such  as  pug-nose,  saddle-uose  and  lorgnette  nose.  The  first  anomaly  is 
characterized  by  flattening  and  broadening  of  the  base  of  the  nose  imme- 
diately below  the  place  where  it  leaves  the  frontal  Iione.  The  second 
anomaly  consists  of  a  sinking  in  of  the  nose  with  a  retraction  of  the 
end,  together  with  an  upward  direction,  so  that  the  axes  of  the  nos- 
trils extend  diagonally  forward  and  upward.  In  the  lorgnette  nose 
(A.  Fournier)  the  lower  nasal  segment  is  unifornih-  depressed  and  seems 
to  come  out  of  the  upper  segment,  much  like  tlie  oculars  of  an  opera  glass. 

Further  permanent  deformities  are  found  in  the  shinbones  in  the 
form  of  thickening  and  tuberosities  of  the  crest  of  the  tibia,  and  in  the 
form  of  the  sword-sheath  shaped  tibia  already  mentioned. 

4.  The  so-called  Hutchimon's  Triad  is  a  group  of  symptoms,  com- 
posed of  changes  in  the  teeth,  ca'cs,  and  ears.  In  the  eyes  of  older  chil- 
dren with  hereditary  syphilis  there  are  frequently  found  leucoma,  spots, 
and  opacities  of  the  cornea,  as  a  sequel  to  a  parenchymatous  keratitis; 
also  changes  in  the  iris,  such  as  synechia  and  irregular  pupils;  as  well 
as  the  spots  on  the  chorioid  that  are  characteristic  of  former  hereditary 
syphihs.  The  liistor}'  usually  discloses  the  existence  of  some  ocular 
trouble  dating  from  earhest  cliildhood.  With  reference  to  the  ear  one 
frequently  obtains  the  historj^  of  an  early  discharge  from  the  ear  or, 
in  some  cases,  of  partial  deafness  without  discharge.  Very  character- 
istic is  a  sudden  deafness  due  to  neuritis  of  the  auditory  nerve.  The 
drum  membranes  may  show  a  great  variety  of  changes. 

It  must  be  remembered  that  irith  the  exception  of  sudden  deafness, 
these  lesions  of  the  organs  of  special  sense  are  not  pathognomonic  of 
hereditary  syphilis.  Parenchymatous  keratitis,  especially,  which  is  usually 
justly  attributed  to  hereditary  syphihs  is  also  found  rarely  in  other 
than  sypliiUtic  children,  particularly  in  those  that  are  debilitated  from 
other  causes,  such  as  tuberculosis.  No  diagnostic  value  can  be  attached 
to  those  stigmata  referable  to  the  organs  of  special  sense,  unless  there  is 
present  other  e\'idence  of  past  hereditary  syphilis. 

The  same  is  true  of  Hutchi7iso7i's  teeth,  an  anomal}'  found  exclu- 
sively in  the  upper  central  incisors  of  the  second  dentition.  Typical 
Hutcliinson's  teeth  always  show  a  single,  rather  superficial,  crescenting, 
broad  notch  in  the  middle  of  the  lower  border,  with  rounded'  corners. 
In  most  cases  the  dentine  is  laid  bare  in  the  centre  of  tliis  crescentic 
notch,  due  to  a  defect  in  the  enamel;  tliis  defect  is  not,  however,  by  any 
means,  a  constant  accompaniment.  Not  rarely  one  finds  these  upper 
central  incisors  either  inclining  toward  or  awaj"  from  one  another:  but 
rarely  are  they  long  enough  to  touch  the  adjacent  teeth.  Tliis  deformity 
certainly  does  occur  in  children  with  hereditary  syphihs  and  Hutcliin- 
son's explanation  for  this  anoniah',  i.e.,  a  nutritional  disturbance  of  the 
dental  germ  due  to  syphilis,  is  doubtless  correct.  But  this  same  harm- 
ful influence  that  syphilis  has  upon  the  dental  germ  can  be  excited  by 


552  THE   DISEASES   OF   CHILDREN 

all  kinds  of  acute  and  chronic  infections  and  constitutional  diseases  if  they 
appear  before  the  eruption  of  the  permanent  teeth.  Thus,  both  Welander 
and  I  have  seen  Hutchinson's  teeth  in  indi^dduals  who  were  positively 
free  from  syphilis,  and   also  in  children  with  early  acquired  syphilis. 

With  reference  to  all  of  these  stigmata,  it  may  be  said  that  those 
alone  have  indisputable  diagnostic  value  that  are  the  result  of  actual 
past  specific  lesions,  such  as  the  scars  on  the  skin  and  mucous  membranes 
and  certain  bone  and  eye  anomaUes;  but  that  many  other  alleged  stig- 
mata, such  as  discharge  from  the  ear,  partial  deafness,  corneal  opacities, 
and  dental  anomahes,  occur  likewise  with  other  diseases  that  are 
accompanied  by  general  weakness  and  lowered  resistance  of  the  youthful 
organism.  An  absolutely  positive  proof  of  former  hereditary  syphilis  is 
found  in  the  radial  scar  formatioyi  on  the  lips. 

Hutclunson's  triad  doubtless  occurs  frequently  in  hereditary  syphi- 
lis; but  it  is  not  a  positive  proof  of  the  existence  of  that  disease.  It 
must  be  mentioned,  further,  that  in  children  who  are  properh^  treated 
in  infancy  these  questionable  symptoms,  especially  that  referable  to  the 
teeth,  only  exceptionally  occur. 

6.    DIAGNOSIS  OF  HEREDITARY  SYPHILIS 

We  have  learned  to  recognize  the  great  diagnostic  value  of  the 
Rontgen  ray  examination  of  the  long  bones  in  fcetal  syphiUs,  since 
Wegner's  osteochondritis  can  easily  be  recognized,  provided  the  foetus 
belongs  to  the  second  half  of  pregnancy. 

It  is  of  the  greatest  prophylactic  and  therapeutic  value  to  make 
the  diagnosis  of  infantile  syphilis  as  early  as  possilbe.  If  we  knew  that 
one  of  the  parents  of  an  apparently  healthy  newborn  child  was  syphi- 
litic, then  we  must  observe  the  cliild  carefully,  so  as  not  to  overlook  a 
possible  specific  infection.  It  must  be  remembered  that  infantile  syph- 
ilis may  run  its  course  without  any  skin  eruption,  and  may  be  recog- 
nized in  practice  only  by  a  dry  coryza,  and  a  striking  pallor,  frequently 
combined  with  some  enlargement  of  the  liver  and  spleen. 

Since,  however,  congenital  syphihs  is  characterized  in  the  great 
majority  of  cases  by  exanthemata,  these  must  take  a  most  prominent 
place  in  making  a  diagnosis.  Since  the  individual  skin  lesions  have  been 
described  before,  it  remains  to  discuss,  at  this  point,  the  differential 
diagnosis  betiveen  the  syphilitic  and  nonsyphilitic  dermatoses  of  infancy. 

Syphilitic  pemphigus  neonatorum  is  distinguished  from  non- 
syphilitic  forms  of  pemphigus,  aside  from  the  predilection  for  the  palms 
and  soles,  by  its  infiltrated  base,  and  by  the  fact  that  the  former  is 
usually  present  at  birth  while  the  latter  does  not  appear  until  a  number 
of  days  after  birth. 

In  distinguisliing  diffuse  hereditary  syphihtic  skin  infiltration  in 
infancy  from   nonspecific  diffuse  inflammatory  processes   it  is  impor- 


SYPHILIS  553 

tant  to  remember  that  all  erythematous  lesions  depending  upon  a 
mechanical,  or  chemical  irritant  show  an  intense,  bright  red  color  while 
that  of  the  sj'pliihtic  dermatoses  is  a  dull  red  with  a  brownish  tint.  The 
same  difference  is  to  be  noted  in  making  the  differential  diagnosis 
between  syphilitic  lesions  and  the  diffuse  reddening  of  the  soles  of  the 
feet  wliich  is  very  frequent  in  atrophic  infants  and  is  dependent  upon 
maceration.  In  the  latter  case,  too,  the  characteristic  induration 
and   the  later  desquamation  is   absent. 

With  reference  to  the  differential  diagnosis  between  eczeyna  inter- 
trigo and  diffuse  sypliiUtic  skin  infiltration,  the  following  points  nmst  be 
kept  in  mind: — The  syphilitic  skin  lesion  never  causes  such  a  brilliant, 
inflammatory,  red  color  as  the  intertriginous  eczema.  Whenever  and 
wherever  present  the  former  has  always  a  suggestion  of  a- copper  red,  or  a 
yellowish  brown  color,  that  the  latter  never  has  it  any  stage.  One  can 
always  tell  upon  careful  examination  by  the  stiffness  of  the  tissues  when 
we  pick  up  an  affected  fold  of  skin,  whether  or  not  there  is  present  a  firm 
infiltrate  as  is  the  case  in  diffuse  skin  syplailis.  In  intertrigo,  it  will 
be  remembered,  there  is  active  hypersemia  and  sw'elling  in  the  papillary 
layer  and  in  the  corium.  These  give  the  impression,  however,  to  the 
palpating  finger,  of  being  soft  and  displaceable,  and  not  of  being  a  firm 
and  unyielding  infiltrate.  The  whole  skin  in  the  region  of  the  nates  and 
about  the  anus  looks  stiffer  and  smoother  in  the  specific  infiltration  than 
in  intertrigo,  has  a  less  briUiant  color  and  does  not  have  the  swollen 
appearance  of  the  intertriginous  dermatitis.  In  the  latter,  too,  espe- 
cially in  the  genito-anal  region  one  never  sees  at  the  height  of  the  process 
any  desquamation.  Scahng  could  only  occur  when  the  acute  inflamma- 
tory redness  and  swelling  have  gone  down,  i.e.,  when  the  acute  process 
has  run  its  course  and  a  restoration  to  the  normal  has  nearly  taken 
place.  In  diffuse  skin  syphiUs  of  these  regions,  however,  eroded  and 
scahng  areas  may  be  situated  side  by  side.  It  is  a  frequent  occurrence 
to  find  the  circumanal  portion  eroded  but  the  skin  of  the  nates  them- 
selves, dry,  smooth,  and  scahng. 

In  the  gluteal  region  of  nonsyphiUtic  children,  an  exanthcm  com- 
posed of  lenticular  spots  may  occur,  the  indi\idual  lesions  of  wiiich 
become  eroded  in  a  short  time  and  very  closely  resemble  moist  sypliihtic 
papules.  These  skin  eruptions  designated  by  Sevestre  and  Jacquet 
"sypliiloides  posterosives,"  are  due  to  maceration  and  are  distinguished 
from  sypliihtic  papules  by  their  brilhant  inflammatory  red  color  and  by 
their  isolated  location  on  the  nates  and  the  posterior  surfaces  of  the  thighs. 

The  locaUzation  of  the  eruption  is  of  value  in  the  differential  diag- 
nosis of  cutaneous  syphihs  of  infancy.  The  efflorescence  and  infiltra- 
tions have  a  predilection  for  regions  of  the  body  that  are  exposed  to 
chemical  and  mechanical  irritations,  therefore  the  lower  half  of  the  body 
and  the  face.     The  color  of  the  skin  is  a  further  point  in  diagnosis.    In 


554  THE   DISEASES   OF   CHILDREN 

the  majority  of  cases  it  is  of  a  light  gray,  while  the  efflorescence  itself 
is  of  a  salmon,  ham,  or  copper  color.  Only  for  a  short  time  during  the 
period  of  eruption  is  there  a  brighter  red  color. 

The  great  value  of  enlargement  of  the  spleen  in  the  newborn  and 
older  infants  has  recently  received  renewed  emphasis  by  Marfan  as  a 
point  in  the  diagnosis  of  hereditary  syphihs  and  is  evident  from  the 
following  figures  estabUshed  by  Pai'rot : 

Weight  of  spleen  in  children  of  5  to  10  days normal 7     grams. 

syphilitic.  .  .  .38  grams. 
Weight  of  spleen  in  children  of  10  to  20  days normal 9.3  grams. 

syphilitic.  ..  .34  grams. 
Weight  of  spleen  in  children  of  20  to  30  days normal 8.3  grams. 

syphilitic ....  18     grams. 

According  to  my  observations,  about  70  per  cent,  of  all  children 
that  have  palpable  spleens  during  the  first  three  months  are  S3'phihtic. 

From  the  standpoint  of  differential  diagnosis,  several  symptoms 
referable  to  individual  organs  should  be  mentioned.  As  far  as  the  nose 
is  concerned,  there  occurs  in  the  newborn  a  condition  in  which  the  nasal 
mucous  membrane  becomes  swollen,  analogous  to  erythema  neonatorum, 
and  is  accompanied  by  a  sHght  snuffle,  a  condition  that  is  physiological. 
There  may  occur  likewise  influenzal,  diphtheritic,  and  gonorrhceal 
corzya  of  infancy. 

The  influenzal  nasal  affection  which  most  frequently  has  to  be  con- 
sidered in  diagnosis,  is  always  accompanied  by  catarrhal  symptoms  of 
the  palate  and  pharynx  that  are  absent  in  the  syphilitic  coryza.  Congenital 
hypertrophy  of  the  tonsils,  too,  can  cause  nasal  obstruction  in  babies. 

As  to  the  mouth  and  pharynx,  it  must  be  remembered  that  in 
infants  syphihtic  efflorescences  and  ulcers  are  among  the  rarest  lesions 
in  these  situations.  By  keeping  in  mind  the  pecuhar  butterfly  shape  of 
the  ulcerations  of  the  hard  palate  that  are  known  as  Bednar's  aphthse 
one  will  never  confuse  them  with  syphilis.  The  constantly  changing 
epithehal  defects  of  the  geographical  tongue  (Landkartenzunge), 
hkewise,  have  no  resemblance  to  specific  lesions. 

The  diagnosis  of  late  hereditary  syphihs  must  be  made  from  the 
characteristic  symptom-complex  above  described.  The  presence  of  a 
complete  Hutchinson's  triad,  together  with  fixed  pupils  and  radial 
scars  on  the  hps,  are  weighty  diagnostic  criteria. 

7.     PROGNOSIS   IN  HEREDITARY   SYPHILIS 
In   18  famihes,   with  syphihtic  parents,  in  which  there  were   161 

pregnancies  A.  Fournier  saw  137   still-births,  i.e.,  85  per  cent.      J.  N. 

Hyde  found  916  deaths  during  the  first  year  of  Hfe  in  1121  syphihtic  births. 
Statistics  from  the  FoundUng  Home  in  Moscow  dating  from  about 

1870  state  that  70  per  cent,  out  of  2038  syphihtic  children  died  during 

their  first  six  months  of  hfe.     Fruhinsholz  had  37  deaths  among  84 


SYPHILIS  555 

congenitally  sypliilitic  children  (68.5  per  cent.,  29  of  these  deaths  occur- 
ring during  the  first  half  year.  Neither  in  private,  nor  in  ambula- 
tory clinical  practice,  however,  does  such  a  condition  exist  as  these 
figures  taken  from  institutions  would  indicate.  Furthermore,  the 
prognosis  in  children  born  with  manifest  syphihs,  especially  those  born 
with  pemphigus,  is  much  more  unfavorable  than  that  of  children  in  whom 
no  evidence  of  syphilis  is  present  till  a  later  period.  Such  syphiUtic 
children  can  be  completely  and  permanently  cured,  according  to  our 
observations,  so  that  they  can  be  reinfected  in  later  years. 

Of  the  early  manifestations  of  hereditary  sypliilis,  the  exanthemata 
and  the  osseous  affections  offer  a  good  prognosis,  except  those  in  which 
pempliigus  is  present  at  birth,  wliile  visceral  affectjons  present  at  birth 
give  a  more  unfavorable  outlook. 

Complicating  affections  and  intercurrent  diseases  have  a  great 
influence  upon  the  fate  of  these  congenitally  syphihtic  children,  espe- 
cially during  the  first  few  months.  Pulmonary  affections  deserve  the 
first  rank,  and  following  them,  gastro-intestinal  diseases.  Sypliilitic 
recurrences  exercise  a  pecuharly  weakening  influence  upon  the  infantile 
organism  during  the  first  few  months  of  hfe,  because  they  very  materi- 
ally lower  the  resistance  of  the  cliild  to  intercurrent  diseases.  It  is 
impossible  to  decide  definitely  whether  or  not  a  pecuUar  predisposition 
for  certain  diseases  exists  in  older  children  with  sj'pliihtic  inheritance. 
The  only  causes  of  death  that  we  have  found  noticeably  frequent  in  these 
cases  are  pulmonary  tuberculosis  and  tuberculous  basilar  meningitis, 
wliicli  must  not  be  confused  with  sypliihtic  meningitis.  The  greatest 
factor  in  determining  the  later  fate  of  these  cases  is  whether  or  not  an 
early  and  rational  therapy  was  instituted  in  the  individual  case. 

From  my  case  histories  I  find  that  the  patients  who  had  no  recur- 
rences were  almost  invariabl}'  those  that  had  been  rigidly  treated  with 
mercury  according  to  our  directions  for  weeks  and  months.  Although 
it  cannot  be  denied  that,  in  spite  of  careful  treatment  of  the  first  period 
of  eruption,  recurrences  are  only  too  frequent,  our  experience  teaches 
that  in  those  children  who  were  properly  treated  at  an  early  stage, 
severe  late  manifestations  in  the  form  of  destructive  gummatous  pro- 
cesses never  occurred.  Hutchinson's  triad,  too,  does  not  occur  under 
these  circumstances. 

On  the  whole  then  the  bad  prognosis  given  to  congenital  sypliilis 
by  obstetricians  and  sypliilologists  must  be  greatly  modified  and 
replaced  by  a  more  favorable  one. 

The  early  affections  of  the  central  nervous  system,  especially  those 
classed  as  meta-  and  paras.vphihtic,  do  not  offer  a  good  prognosis  as  to 
permanent  cure;  while  the  disturbances  of  locomotion  due  to  bone 
lesions,  as  well  as  the  early  osseous  manifestations,  jield  rapidly  to 
rational  treatment  and  are  completely  cured. 


556  THE   DISEASES   OF   CHILDREN 

Recurrences  of  hereditary  sypliilis  are  much  more  frequent  in 
untreated  cases;  85  per  cent,  of  all  my  cases  of  congenital  sypliihs  of 
the  fourth  to  the  sixth  month  were  cases  that  had  not  been  treated 
before,  and  were  brought  for  examination  on  account  of  a  recurrence. 
The  prognosis  as  to  permanent  cure  in  these  cases  is  somewhat  less 
favorable  than  in  those  cases  that  are  pToperly  treated  from  the  start. 
Those  that  are  not  treated  early  are  the  ones,  for  the  most  part,  that 
later  give  evidence  of  late  hereditary  syphihs. 

What  prognosis  shall  we  give  to  cases  of  late  hereditary  syphilis? 
Skin  and  bone  manifestations  as  such  are  doubtless  curable  in  the 
majority  of  cases,  and  yet  they  are  much  more  difficult  to  eradicate 
than  those  of  early  syphilis.  In  general,  it  may  be  said  that  those 
afflicted  with  such  manifestations  are  to  be  considered  below  par  vnth 
reference  to  their  permanent  state  of  health,  and,  in  later  life,  they  are 
candidates  for  parasyphilitic  affections  of  the  vascular  and  nervous 
systems. 

Even  more  than  in  the  case  of  the  gummatous  process  of  late  hered- 
itary syphilis  the  parasypliihtic  affections  of  tliis  period  depend  upon 
the  treatment  of  the  early  manifestations  of  the  disease.  In  my  exten- 
sive material,  tabes  and  paralysis  occurred  only  in  cases  in  which  the 
early  syphilis  was  inadequately  treated. 

ACQUIRED  SYPHILIS   OF  CHILDHOOD 

There  is  no  essential  difference  between  the  acquired  syphilis  of 
the  child  and  that  of  the  adult.  Well  developed  cases  of  acquired  syphiUs 
in  children  under  observation  from  the  beginning,  show  a  primary 
lesion  at  the  point  of  infection,  wliich  is  followed  after  the  proper  interval 
by  the  development  of  indolent  buboes  and  of  a  universal  skin  eruption. 
The  majority  of  these  cases,  however,  do  not  come  under  observation 
at  the  start,  so  that  there  is  usually  nothing  left  of  the  primary 
lesion.  Moreover,  the  latter  does  not  always  appear  as  a  characteristic 
Hunterian  induration,  but  is  frequently  a  simple  papule.  It  is  rare, 
too,  that  one  sees  the  first  exanthem.  In  our  own  ambulatory  clinic 
these  cliildren  with  acquired  syphiHs  present  themselves  as  a  rule  with 
condylomata.  Since  the  primary  lesion  and  the  skin  eruptions  are  very 
frequently  not  well  marked  in  children,  they  are  apt  to  be  overlooked, 
while  the  appearance  of  extensive  condylomata  causes  the  parents  to 
seek  medical  aid.  In  locaUties  where  sypliihs  is  endemic  tliis  is  not  the 
case,  according  to  the  reports  of  L.  Gliick  of  Sarajewo. 

Contagion  may  take  place  in  children  just  as  in  adults,  i.e.,  through 
venereal  contact,  through  accidental  transmission  to  the  surface  of  the 
body,  and  through  contact  ^\■ith  unclean  instruments  in  the  hands  of 
the  physician.  The  following  methods  of  infection  must  be  considered 
as  peculiar  to  children:  (1)  transmission  at  the  time  of  birth  from 
an   actively   syphilitic   mother;    (2)    transmission    through    the    act    of 


SYPHILIS  5;57 

nursing;  (3)  transmission  through  various  measures  employed  in  the 
care  of  children. 

A.  Fournier  denies  the  possibihty  of  an  intrapartum  infection, 
believing  in  the  validity  of  Profeta's  law  with  reference  to  the  immunity 
of  a  syphihtic  mother.  Nevertheless,  six  well  authenticated  cases  of 
such  infection  have  been  described  up  to  the  present  time. 

Of  greater  importance  is  the  possibihty  of  infection  through  nurs- 
ing. It  is  evident  that  a  nurse  with  virulent  sypliihtic  lesions  on  the 
breast  can  infect  the  child  that  nurses  her.  In  that  case  a  primary  lesion 
forms  on  the  hps,  more  rarely  at  the  entrance  to  the  nostrils.  It  is  also 
conceivable  that  a  nurse  can  transmit  syphihs  to  a  child,  without  her- 
self being  syphihtic  if  she  nurses  alternately  at  the  same  breast  a  syph- 
ilitic and  a  nonsypliilitic  child.  It  has  happened,  for  example,  that  the 
sahva  of  a  syphihtic  cliild  served  as  the  bearer  of  contagion  to  a  well 
baby  when  the  latter  was  given  the  same  breast,  without  sufficient 
cleansing,  immediatelj'  after  the  former  had  nursed.  The  presence  of 
primary  lesions  simultaneously  on  the  breast  of  the  nurse  and  in  the 
mouth  of  the  baby  can  be  explained  only  in  tliis  manner. 

In  those  infections  occurring  as  a  result  of  the  usual  attentions 
given  to  infants  and  children,  chance  plays  a  prominent  part.  The 
primary  lesion  may  be  situated  anywhere  on  the  surface  of  the  skin  or 
mucous  membranes.  By  far  the  most  frequent  seat  in  these  cases  is  the 
mucous  membrane  of  the  hps,  especially  of  the  lower  Up,  because  of  the 
fact  that  feeding  and  caressing  are  the  most  prominent  causes.  Chancre 
of  the  eyehds,  too,  has  been  seen  in  children  as  a  result  of  kissing. 

The  point  of  infection  is  only  rarely  the  genitaha,  and  then  much 
more  frequently  in  girls  than  in  boys.  This  method  of  contagion  is 
sometimes  the  result  of  violence  on  the  part  of  an  individual  who  holds 
a  view  that  is  wide  spread,  that  transmission  of  liis  disease  to  a  young 
virgin  will  cure  liis  own  syphilis  (A.  Fournier).  The  finding  of  a  pri- 
mary lesion  on  the  genitaha  of  a  child  should  always  be  reported  to  the 
poUce,  since  it  is  probably  the  result  of  a  criminal  attack. 

As  to  transmission  liy  the  physician  in  lais  professional  capacity, 
this  had  to  be  considered  formerly,  as  occurring  through  vaccination. 
Occasionally,  rituahstic  circumcision  \nth  accompanjTng  sucking  of  the 
wound  by  the  operator  has  led  to  infection  in  the  cliild. 

Our  own  experience  and  examination  of  the  hterature  leads  us  to 
say  that  the  site  of  the  primary  lesion  is  most  frequently  on  the  hps, 
less  frequently  on  the  face  or  neck,  and  still  less  frequentlj'  on  the 
perineum,  the  abdomen  and  the  genitaha.  The  least  frequent  of  all  in 
cliildhood  is  a  chancre  of  the  tonsil  or  of  the  tongue. 

Chancre  of  the  tonsil  is,  however,  relatively  more  frequent  in  cliil- 
dren  than  in  adults,  and  is  characterized  by  moderate  enlargement  of 
the   tonsil  together  with  ulceration  of  its  surface  accompanied  by  a 


558  THE    DISEASES   OF   CHILDREN 

grayish,  moist  coating  that  has  often  led  to  a  diagnosis  of  diphtheria. 
The  submaxillary  and  cervical  lymph-nodes  are  always  enlarged,  always 
bilaterally,  though  the  chancre  is  of  one  tonsil  only. 

The  course  of  acquired  syphihs  in  childhood  is  generally  a  mild 
one,  even  in  infancy.  A.  Fournier  has  reported  cases  of  acquired  syph- 
ihs in  infancy,  in  which,  on  account  of  the  enormous  development  of 
condylomata  of  the  mucous  membrane  the  nutrition  was  seriously  im- 
paired and  a  cachectic  condition  was  produced.  I  myself  have  never 
seen  such   cases. 

In  older  cliildren,  according  to  my  experience,  the  first  skin  erup- 
tion causes  no  constitutional  disturbances.  I  agree  with  Heubner  who 
has  called  attention  to  the  infrequency  of  a  general  eruption  in  ac- 
quired sypliilis  in  cliildren.  Among  52  cases  in  my  own  material,  both 
institutional  and  private,  I  could  find  a  general  eruption  in  only  13 
cases,  wliile  all  of  the  cliildren  showed  at  some  time  during  the  period  they 
were  under  observation,  moist  condylomata  in  one  location  or  another. 

The  exanthemata  observed  by  me  as  the  first  in  each  case  were 
invariably  macular.  Twice  I  saw  an  orbicular  sypliihde  as  an  erup- 
tion occurring  during  a  recurrence.  In  the  case  of  endemic  syphilis, 
according  to  the  observations  of  Gliick,  somewhat  different  conditions 
prevail. 

The  great  frequency  of  moist  condylomata,  with  a  predilection 
for  the  mouth  and  pharynx,  the  genitaUa,  the  anus  and  the  scrotum, 
is  recognized  by  all  (see  Plate  25).  Gliick  has  frequently  seen  moist 
papules  on  the  nasal  mucous  membrane  as  well. 

The  indolent  polyadenitis  of  sypliihs  is  always  very  well  marked 
in  acquired  syphihs  of  cliildhood.  The  submaxillary  nodes  usually 
show  the  greatest  enlargement  even  if  there  is  no  induration  found  on 
the  hps,  probably  due  to  the  great  frequency  of  the  primary  infection 
of  the  lips  in  the  form  of  a  papule. 

Heubner  has  pointed  out  the  lack  of  resistance  of  these  children 
with  acquired  sypliihs  to  other  infectious  diseases.  There  seems  also, 
according  to  my  observations,  a  pecuhar  predisposition  to  later  tubercu- 
losis, much  as  is  the  case  of  children  with  hereditary  syphihs. 

Severe  recurrences  are  less  frequent  in  the  acquired  than  in  the 
hereditary  form  of  sypliihs,  and  the  reappearances  of  the  former  are 
nearly  always  mild  and  yield  most  reachly  to  proper  treatment. 

It  is  often  necessary  to  make  a  differential  diagnosis  between  hered- 
itary and  acquired  syphihs  in  a  child.  The  decision  is  often  very  im- 
portant because  the  source  of  infection  must  be  made  out  and  removed. 
If  we  have  to  do  -with  a  hereditary  transmission,  then  the  diseased  par- 
ent must  be  treated;  if  on  the  other  hand  the  case  is  one  of  contact 
infection,  then  the  bearer  of  infection  must  be  discovered,  eliminated 
from    the    household,    and    treated.      If   a    primary   sclerosis    can   be 


SYPHILIS  559 

demonstrated  there  is,  manifestly,  no  doubt  that  the  disease  is  of  the 
acquired  form;  while  diffuse  infiltrations  of  the  skin  and  mucous  mem- 
branes, i.e.,  diffuse  palmar  and  plantar  syphilide,  infiltration  and  scars 
of  the  lips,  diffuse  rhinitis  and  nasal  deformities  during  the  first  period 
of  hfe,  point  unquahfiedly  to  hereditary  syphilis.  The  differential 
diagnosis  is  generally  more  easily  made  during  infancy  than  at  a  later 
period.  The  greatest  difficulty  arises  in  the  case  of  children  more  than 
a  year  old  with  condylomata,  because  these  can  occur  equally  well  as  a 
recurrence  of  hereditary  sypliilis,  or  as  a  new  manifestation  of  the  ac- 
quired form.  In  many  cases  the  liistory  of  the  parent  will  disclose  the 
hereditary  nature  of  the  disease.  In  dispensary  cases  such  e\ddence  is 
very  frequently  not  obtainable;  then  the  general  condition  of  the  child 
must  decide.  It  is  wholly  improbable  that  a  child  with  hereditary 
S5^phihs,  untreated  during  the  first  year  of  Ufe,  is  free  from  .specific 
stigmata.  These  are  usually  abnormahties  of  the  cranium  and  of  the 
nasal  skeleton  as  already  mentioned.  If  these  are  absent  and  the  gen- 
eral condition  of  the  cliild  is  good,  then  one  can  diagnosticate  acquired 
sj-pliihs  with  certainty. 

The  presence  of  a  syphihtic  roseola  on  the  trunk,  speaks  positively 
for  the  acquired  type,  as  it  never  occurs  there  in  the  hereditary  form. 
On  the  other  hand,  various  dis.?eminated  exanthemata,  such  as  the 
small  papulse  and  orbicular  forms,  may  occur  during  the  second  year 
of  Ufe  as  manifestations  of  a  recurrence  of  herechtary  syphihs,  although 
this  is  an  extremely  rare  occurrence,  and  is  never  unaccompanied  by 
other  stigmata  of  the  hereditary  form  of  the  disease,  especially  in  the 
nervous  system. 

The  differential  diagnosis  between  late  hereditary  sypliihs  and 
the  tertiary  stage  of  acquired  .sypliihs  in  children  is  much  more  diffi- 
cult. 7  knoio  of  but  one  pathognomonic  symptom:  the  presence  of  radial 
scars  on  the  lips.  This  occurs  only  in  hereditary  syphilis.  Hutchinson's 
triad  is  found  also  in  tertiary  acquired  syphihs. 

SYPHILIS  AND  INFANT  FEEDING 
It  may  be  stated,  first  of  all,  that  artificial  feeding  of  congenitally 
sypluUtic  infants  born  at  full  term  is  attended  by  only  slightly  greater 
risk  than  in  those  that  arc  free  from  this  disease.  In  general,  in  order 
to  avoid  transmission  to  the  person  who  nurses  it,  the  infant  should  be 
artificially  fed.  Most  authors,  in  deference  to  Colles'  law,  permit  the 
mother  who  is  free  from  sypliihs  to  nurse  her  diseased  cliild.  The  num- 
ber of  exceptions  to  this  law  have  become  so  great,  that  Ogihde,  Finger, 
and  myself,  hold  the  A-iew  that  infants  with  infectious  lesions  should  not 
be  permitted,  off  hand,  to  nur.se  their  mothers.  Since  75  per  cent,  of 
the  exceptions  to  CoUes'  law  were  primiparse,  I  might  maintain  that  the 
latter  should  never  be  exposed  to  the  danger  of  infection  which  nursing 


560  THE    DISEASES   OF   CHILDREN 

carries  with  it;  on  the  other  hand,  I  have  greater  faith  in  CoUes' 
immunity  in  the  ease  of  multipara;  and  permit  these  to  nurse  their 
sj'pliilitic  babies. 

What  shall  be  done  in  the  case  of  an  actively  syphilitic  woman  who 
gives  birth  to  a  healthy  baby?  Profeta's  law  claims  immunity  against 
specific  infection  for  the  offspring  of  a  syphihtic  woman,  and  Ehrlich's 
investigations  show  that  the  congenital  immunity  of  the  offspring  of 
highly  immunized  animals,  i.e.,  mothers,  is  heightened  bj'  suckhng  the 
mother.  The  healthy  child  of  a  syjihihtic  mother  has  therefore  only  a 
very  slight  chance  of  becoming  specifically  infected  during  the  first  few 
months  of  life,  even  if  it  is  nursed  by  its  mother.  A  cliild  that  appears 
healthy  at  birth  may  therefore  be  permitted  to  nurse  the  syphilitic 
mother.  The  mother  must  be  treated  with  mercury,  and  the  greatest 
care  must  be  exercised,  as,  for  example,  by  permitting  no  caressing,  so 
that  the  chances  of  transmission  of  the  disease  to  the  child  will  be 
reduced  to  a   minimum. 

The  question  will  arise  whether  a  child  with  syphihtic  heredity 
shall  be  allowed  to  have  a  wet-nurse.  The  question  may  be  considered 
under  two  conditions:  (1)  What  shall  be  done  if  the  child  has  symp- 
toms of  syplaiUs?  (2)  What  shall  be  done  in  the  case  of  infants  that  are 
born  health3^  but  have  sj-phihtic  parents? 

In  the  former  case  the  employment  of  a  wet-nurse  must  be  for- 
bidden, even  if  after  being  fully  informed  of  tlie  nature  of  the  disease 
and  its  contagiousness  she  is  ^\ilHng  to  nurse  the  baby.  One  would 
have  to  consider  here  not  only  the  infection  of  the  nufse,  but  also  the 
possibihty  of  a  further  spreading  of  the  disease  through  her.  The  only 
condition  under  which  it  would  be  permissible  to  give  the  breast  to  a 
manifestly  sypliihtic  newborn  cliild  would  be  that  of  producing  a  wet- 
nurse  who  had  recently  had  syphilis. 

In  the  second  case,  one  can  put  a  healthy  newborn  babe  of  a  form- 
erly sypliihtic  father  to  the  breast  of  a  wet-nurse  without  fear  of  con- 
tagion, but  the  child  should  be  most  carefully  watched  so  that  it  can  be 
taken  from  the  breast  at  once  and  treated  with  mercury  if  it  shows  the 
first  symptoms  suspicious  of  the  disease. 

If  unfortunately  a  wet-nurse  becomes  infected  with  syphilis  from 
the  child  she  is  nursing,  it  is  the  duty  of  the  family  to  have  her  undergo 
antisyphiUtic  treatment  at  once,  and  to  safeguard  others  by  preventing 
her  from  minghng  freely  ■uith  the  outer  world.  She  must  either  be  kept 
isolated  and  treated  in  the  household  in  which  she  became  infected,  in 
which  case  she  may  continue  to  nurse  the  cloild  that  infected  her; 
or  she  must  be  treated  in  an  institution  and  the  child  must  be  fed 
artificially.     The  former  course  is  to  be  preferred. 

It  is  self-evident  that  one  must  be  mindful  of  the  possibihty  of 
others  in   the   household   being  infected   by   the   syphihtic   cliild,   and 


I'T.ATE  20. 


I.     Syphilis  hereditaria  tarda  of  the  left  uhia  in  a  10-year-oM  boy. 
ulna  due  to  ossification  of  a  periosteal  proliferation. 
II.     Syphilis  hereditaria  tarda.     Ten-year-old  girl. 


Spint-lle-forni  cnlargeaicut  at  luiJdle  of 


SYPHILIS  561 

appropriate  prophylactic  measures  must  be  taken.  The  view  that  /(as 
been  expressed  many  times  that  congenital  syphilis  is  less  contagious  than 
the  acquired  form  is  erroneous. 

The  possibility  of  infection  of  a  healthy  child  through  a  wet-nurse 
who  is  sypliiUtic,  either  through  pre\'iously  aciiuired  disease  or  through 
disease  acquired  in  some  manner  during  the  nursing  period,  must  still 
be  considered.  It  is  always  a  peculiarly  embarrassing  situation  for  the 
physician  to  find  symptoms  of  syphiUs  in  a  wet-nurse  employed  for  a 
child  under  liis  care.  The  nurse  will  always  maintain  that  she  was 
infected  by  the  child,  and  this  question  must  be  settled  first.  From  an 
examination  of  the  child,  or  still  more  from  the  preceding  observation 
of  the  child,  it  is  usuall}'  not  difficult  to  decide  whether  the  child  is 
syphihtic.  The  location  and  nature  of  the  .syphilitic  manifestations  in 
the  nurse  will,  evidently,  give  valuable  information  as  to  whether  the 
child  was  the  source  of  her  infection.  One  would  be  slow  to  accept  the 
probability  of  transmission  from  the  infant  to  the  nurse,  unless  the 
latter  had  a  chancre  on  the  breast.  The  physician  who  examined  the  nurse 
before  she  was  employed  is  very  apt  to  Ije  blamed  for  carelessness  in 
his  examination,  although  she  may  have  been  at  that  time  in  the  first 
period  of  incubation,  or  in  the  latent  period  of  a  previously  contracted 
disease,  both  of  which  conditions,  of  course,  giving  rise  to  no  recogniz- 
able signs  of  the  disease.  In  any  event  the  nurse  must  be  dismissed  at 
once.  If  the  cliild  has  not  yet  become  infected,  it  will  probably  remain 
free  from  syphilis  unless  it  is  already  in  the  incubation  period.  If  it  is 
infected  or  is  in  the  period  of  incubation,  then  the  removal  of  the  nurse, 
who  ought  to  be  given  thorough  antisyphihtic  treatment,  will,  at  least, 
lower  by  one  the  number  of  carriers  of  infection  in  that  particular  fam- 
ily. The  child  taken  from  the  breast  of  a  syphihtic  woman  must  under 
no  circumstances  be  given  to  another  woman  to  nurse,  but  must  be 
fed  artificially.  If  the  child  should  not  thrive  on  artificial  feeding 
after  a  numlx-r  of  weeks,  and  still  remains  free  from  sypliilis,  then 
another  nurse  may  be  employed. 

Antenatal  Prophylaxis.— One  should  endeavor  to  prevent  the 
birth  of  sypliihtic  children  by  appropriate  treatment  of  the  syphihtic 
parents,  either  before,  or  after  marriage.  Healthy  children,  further, 
must  be  protected  against  infection  by  a  rigid  surveillance  of  wet-nurses, 
and  by  all  those  measures  that  tend  to  prevent  contact  infection  from 
one  person  to  another.  It  is  evident  that  a  consideration  of  the  "pro- 
tection of  the  child  against  sypliihs"  should  include  also  the  "protec- 
tion of  the  wet-nurse  against  sypluhs  from  the  child  she  nurses." 

With  reference  to  the  prevention  of  syphihtic  births,  we  should 
begin,  first  of  all,  with  marriage.  The  important  point  here  is  to  deter- 
mine the  state  of  health  of  the  person  who  is  about  to  marry.  It 
devolves  upon  the  family  physician  to  insist  upon  a  frank  confession  on 

11—36 


562  THE    DISEASES   OF   CHILDREN 

the  part  of  the  candidate  for  matrimony  as  to  whether  he  has  had 
sypMhs,  at  what  time,  and  with  what  symptoms,  and  not  to  permit 
marriage  until  at  least  four  years  have  elapsed  since  the  primary  infec- 
tion, during  three  years  of  which  he  has  been  treated  systematically, 
and  during  the  last  six  months,  at  least,  of  this  time  he  should  have  been 
wholly  free  from  symptoms  of  syphihs.  Even  with  all  of  these  pre- 
cautions, too  many  syphilitic  children  will  still  be  born  to  such  par- 
ents. The  only  absolute  protection  against  hereditary  syphihs  would 
be  the  prevention  of  the  marriage  of  all  men  who  have  ever  been  syphi- 
litic and  their  strict  adherence  to  coitus  condomatus  in  general. 

In  those  rare  cases  where  the  woman  was  syphihtic  before  marriage, 
a  much  longer  proliibition  period  should  be  enjoined,  because  the  pos- 
sibihty  of  transmitting  the  disease  to  the  offspring  extends  over  a  longer 
period  of  time  in  the  case  of  the  mother  than  of  the  father.  Though  it 
is  permissible  to  allow  marriage  to  the  syphihtic  if  there  has  been  a  rigid 
enforcement  of  the  above-named  conditions,  one  should  not  encourage  it. 

Different  conditions  are  presented  to  the  physician  if  marriage 
has  already  taken  place  with  one  of  the  contracting  parties  sypliihtic. 
If  the  man  is  in  a  period  of  latency,  the  woman  free  from  sypliihs,  and 
the  offspring  nevertheless  sypliihtic,  no  matter  whether  born  as  the 
result  of  abortion,  as  still-births,  or  as  manifestly  syphihtic  infants,  the 
husband  should  undergo  energetic  treatment,  to  prevent  the  birth  of 
other  infected  children.  I  do  not  consider  it  necessary  to  treat  the  mother 
in  whom  the  physician  has  never  seen  evidence  of  syphiUs,  in  the 
above  circumstances,  and  I  forbid  the  husband  to  have  any  intercourse 
with  his  wife  during  the  period  of  treatment  with  mercury.  If  the  wife 
becomes  pregnant  after  the  husband  has  completed  this  energetic  treat- 
ment with  mercury,  it  is  still  a  matter  of  dispute  whether  she,  too, 
should  be  required  to  take  a  hke  treatment.  Those  who  bcheve  that 
syphiUs  can  be  transmitted  only  by  the  mother,  would  treat  the  preg- 
nant wife  even  if  she  had  never  had  symptoms  of  the  disease  (Matze- 
nauer,  Mracek);  I  consider  such  a  course  superfluous.  If,  however, 
the  pregnant  woman  had  shown  symptoms  of  specific  infection  either 
before  or  during  her  pregnancy,  or  if  there  is  evidence  of  such  infection 
from  the  liistory,  then  she  must  be  given  a  most  energetic  mercurial 
treatment.  Riehl  has  recommended  for  this  purpose  combining  the 
constitutional  treatment  of  the  mother  during  pregnancy  with  the  local 
use  of  vaginal  suppositories  each  containing  mercurial  ointment  1.0 
Gni.  (15  gr.),  cocoa  butter  1.0-2.0  Gm.  (15-30  gr.),  a  procedure  that 
has  the  warmest  approval  of  Vomer. 

If  the  date  of  primary  infection  of  the  husband  is  far  removed  and 
the  wife,  who  has  remained  and  is  now  free  from  syphihs,  becomes  preg- 
nant, it  is  advisable  to  omit  all  antisyphiUtic  treatment  of  both  parties 
and  quietly  await  the  termination  of  pregnancy. 


SYPHILIS  563 

TREATMENT  OF  SYPHILIS  IN  CHILDHOOD 

1.  Treatment  of  Early  Hereditary  Syphilis. — Shall  every  cliild 
of  syphilitic  parentage  be  treated,  no  matter  whethei  it  has  symptoms 
of  syphilis  or  not,  at  birth?  I  would  answer  this  question  by  saying 
that  only  those  should  be  treated  that  have  evident  syphilitic  symp- 
toms, among  which  are  to  be  classed  not  only  skin  affections,  but  also 
diseases  of  the  nose,  of  the  bones  and  of  the  viscera.  A.  P'ournier  holds 
the  view  that  the  healthy  child  of  a  recently  syphilitic)  mother  should 
certainly  be  treated,  but  that  the  healthy  child  of  a  mother  with  an  older 
specific  infection  need  not  be  treated.  /  recognize  but  one  indication  for 
antisyphilitic  treatment  in  the  newborn  and  young  infant,  i.e.,  manifest 
syphilis,  no  matter  whence  it  comes. 

The  only  effective  therapy  of  early  hereditary  syphilis  is  admin- 
istration of  the  preparations  of  mercury.  In  the  front  rank  should  be 
placed  the  internal  use  of  the  yellow  iodide  of  mercury  (protiodide), 
introduced  as  a  therapeutic  agent  in  infantile  sypliihs  by  Forster  and 
L.  M.  Politzer,  and  now  used  exclusively  at  my  cUnic.     We  prescribe: 

Protiod.  liydrarg 0.1 grs.  iss 

Pulv.  acaciEE 5 . 00 3  i,  grs.  xv 

in  dos.  10-15  Gm.  (2t-4  dr.),  stirred  in  milk,  and  give  our  cliildren  3  such 
doses  a  day,  until  all  symptoms  of  syphiUs  have  disappeared;  after  this 
wc  continue  2  pills  for  two  weeks  and  then  one  pill  for  the  same  length 
of  time.  If  extensive  crusty  and  moist  skin  lesions  are  present,  we  use 
in  addition  baths  of  bichloride  of  mercury  1  Gm.  (15  gr.)  to  20  htres 
(5  gallons)  of  water  and  leave  the  cliild  in  tliis  for  15  to  20  minutes. 

Other  preparations  of  mercury  are  calomel,  hydrarg.  tannic,  oxydul., 
or  a  preparation  much  used  in  England,  hydrargyrum  cum  creta  (gray 
powder),  all  of  wliich,  however,  are  not  to  be  compared  with  the  pro- 
tiodide in  their  effectiveness  aganist  sj'phiHs. 

French  physicians,  as  for  example  at  the  Chnique  Tarnier,  use  very 
extensively,  the  so-called  liquor  ran  Swieten,  made  as  follows: 

Hydrarg.  chlor.  corro.siv 1.0 gr.  xv 

Alcohol 100.0 5  iii 

Aqua  dest 900 .0 5  xxx 

10-.30  gtt.  per  day. 

The  use  of  tliis  preparation  is  all  the  more  unjustifiable,  in  that  it 
contains  10  per  cent,  alcohol. 

Mercurial  inunctions,  too,  arc  frequently  used  in  treating  sypliilis 
in  infancy.  We  have  almost  wholly  abandoned  its  use  during  the  first 
few  months,  as  has  also  Finkelstein,  but  use  it  in  preference  to  other 
preparations  in  syphilitic  recurrences  during  the  second  and  third  years, 
especially  in  affections  of  the  central  nervous  system.  For  inunction 
we   may  use  the  mercurial  ointment,   10  per  cent,   colloidal  mercury, 


564  THE    DISEASES    OF   CHILDREN 

mercury  resorbin,  or  mercury  vasogen.  One  gram  of  the  ointment  is 
rubbed  in  daily  for  5  minutes.  The  skin  that  is  to  be  rubbed  should  first 
be  waslred  with  warm  water  and  soap,  and  after  the  rubbing  it  is  to  be 
covered  with  a  layer  of  absorbent  gauze.  In  order  to  avoid  irritation,  a 
new  area  of  skin  must  be  chosen  each  day  for  the  inunction. 

The  following  arrangement  for  10  days  is  a  good  one  for  children: 

First  day,  left  side  of  chest.  Sixth  day,  inner  surface  of  left  thigh. 

Second  day,  right  side  of  chest.  Seventh  day,  right  calf. 

Third  day,  left  side  of  abdomen.  Eighth  day,  left  calf. 

Fourth  day,  riglit  side  of  abdomen.         Ninth  day,  right  arm. 
Fifth  day,  inner  surface  of  right  thigh.  Tenth  day,  left  arm. 

The  presence  of  bullous  and  crusty  skin  lesions,  as  well  as  bone  af- 
fections contraindicates  the  use  of  the  inunctions. 

Subcutaneous  injection  of  soluble  mercury  preparations  offers  no 
advantage  over  the  other  methods.  The  intravenous  injection  of  sol- 
uble mercurial  salts  is  still  less  to  be  recommended.  The  method  intro- 
duced by  Welander  of  using  a  little  laag  and  the  merkolintschurz  of 
Blaschko  patterned  after  it,  I  have  abandoned  as  less  efficient  than 
other  methods.  The  appHcation  of  large  pieces  of  various  mercurial 
plasters  to  the  back,  as  recommended  by  Unna  and  E.  Lang,  is  a  very 
effective  method,  but  is  apt  to  produce  an  undesirable  skin  irritation. 

Since  there  is  more  at  stake  in  the  treatment  of  hereditary  sypMhs 
than  of  the  acquired  form,  as  the  former  is  usually  a  matter  of  life  and 
death,  it  is  desirable  to  use  only  one  method  of  treatment  wliich  we 
know  will  produce  the  desired  result  without  causing  other  disturbances. 
From  this  standpoint  I  can  recommend  only  the  internal  administra- 
tion of  the  protiodide,  and  the  inunction  method,  under  the  conchtions 
described.  The  protiodide  is  especially  well  borne  by  the  gastro-intes- 
tinal  tract  of  children  and  only  very  exceptionally  causes  transient 
diarrhoea.  /  have,  myself,  never  had  occasion  to  stop,  nor  interrupt,  the 
treatment  ivith  protiodide. 

A  really  effective  treatment  of  the  cliild  by  means  of  the  admin- 
istration of  mercury  to  the  nursing  mother  is  inconceivable,  especially 
since  the  latest  investigations  of  Somma  have  shown  that  mercury  is 
not  excreted  with  the  milk. 

The  child  requires  no  special  care  of  the  mouth  during  treatment 
with  mercury,  as  the  adult  does.  SaUvation  never  occurs,  to  say  noth- 
ing of  mercurial  stomatitis.  The  administration  of  mercury  not  only 
rapidly  drives  away  all  symptoms  of  syphilis  in  those  infants,  but 
likewise  raises,  noticeably,  the  hsemoglobin  content  of  the  blood. 

The  iodides  are  useless  in  the  treatment  of  infantile  sypMhs.  In 
sypliihtic  recurrence  in  the  second  or  third  year  in  the  form  of  affections 
of  the  central  nervous  system  and  of  the  bones  the  iodides  can  be  used 
internall}'  as  an  aid  to  the  use  of  mercury  by  inunction.     About  0.3-0.5 


SYPHILIS  565 

Gm.  (4J-7J  gr.)  of  sodium  iodide  should  be  given  daily.  The  sub- 
cutaneous and  intramuscuhir  injections  of  iodipin  can  bo  dispensed  with 
entirely.  The  treatment  of  individual  important  local  affections  must 
not  be  neglected. 

Treatment  of  Rhinitis. — Local  treatment  is  demanded  if  there 
is  considerable  nasal  obstruction  and  much  secretion.  I  have  had  the 
most  satisfactory  results  with  cotton  tampons  attached  to  a  string, 
painted  over  with  a  5  per  cent,  red  precipitate  ointment.  Such  tam- 
pons are  placed  alternately  in  one  nostril  and  then  the  other,  being  left 
for  one  hour  at  a  time.  The  nostril  is  first  cleansed  each  time,  with  a 
moist  cotton  swab.  Excoriations  in  the  vicinity  of  the  nostrils  are 
powdered  with  the  following: 

R     Calomel 5 giss 

Zinci  oxidi 5 3  iss 

Amyli 50 3  x 

If  the  nose  is  so  obstructed  that  breathing  and  nursing  are  inter- 
fered with,  it  is  well  to  put  several  drops  of  a  1-5000  adrenalin  solu- 
tion, in  weak  boric  acid,  into  the  nostrils  a  number  of  times  a  day.  If 
there  is  a  profuse  purulent  secretion,  an  effective  remedy  is  found  in 
the  instillation  into  the  nostrils  of  10  per  cent,  perhydrol  (Merck)  or 
painting  vnih  2  per  cent,  creosote  glycerin. 

Treatment  of  the  Skin  Affections. — This  differs  in  no  way  from 
the  customary  treatment  of  skin  lesions  in  acquired  syphilis.  Moist 
condylomata  in  the  genito-anal  region  are  best  dusted  with  calomel 
powder  and  then  covered  with  a  layer  of  absorbent  gauze  that  has  been 
dipped  in  5  per  cent,  salt  solution.  After  the  new  skin  has  formed,  the 
hypertrophic  papules  are  to  be  covered  with  emplastrum  cinereum. 
Unna's  mercury-guttapercha  plaster  mull,  as  made  by  Beiersdorf  of 
Hamburg,  is  pecuUarly  adapted  to  the  local  treatment  of  syphilitic 
skin  lesions  in  cliildren.  Encasing  the  end  phalanges  of  the  fingers,  or 
toes,  in  paronychia,  with  this  plaster,  gives  extraordinarily  good  results. 

It  is  best  not  to  give  any  local  treatment  for  the  crusty,  wide-spread 
sypliihde.  The  bichloride  bath,  as  an  addition  to  the  internal  treatment, 
is  of  great  value  in  this  condition. 

Ulcerations  are  to  be  covered  with  subhmate  gauze  and  then 
collemplastrum  cinereum  spread  on  tliis. 

Diffuse  Skin  Infiltrations  require  no  local  treatment,  but  comph- 
cating  skin  inflammations  do.  Furuncles  and  abscesses  must  be  opened 
as  soon  as  possible,  and  erosions  of  the  umbiUcus  must  be  treated  prop- 
erly, best  by  cauterization  with  silver  nitrate.  It  is  a  general  rule  that 
collections  of  pus,  wherever  localized,  must  be  removed  without  delay. 

Treatment  of  the  Lesions  of  Mucous  Membranes. — Since  we  have 
discussed  nasal  affections,  and  since  other  lesions  of  the  mucous  mem- 
branes are  very  rare  in  early  hereditary  syphilis,  there  is  not    nnich 


566  THE   DISEASES   OF   CHILDREN 

more  to  be  said  here.  Affections  of  the  mouth  and  pharynx  require 
painting  wdth  a  1  per  cent,  solution  of  bichloride  of  mercury,  or  a  10 
per  cent,  silver  nitrate  solution.  Local  treatment  of  the  laryngeal 
affections  is  impossible  in  early  cliildhood. 

Treatment  of  Bone  Lesions. — Local  treatment  is  not  necessary. 
The  bone  swelhngs  and  the  accompanying  disturbances  of  motion  are 
among  the  most  satisfactory  lesions  in  hereditary  syphilis  from  a  thera- 
peutic standpoint.  Phalangitis  alone  reacts  a  little  less  rapidly  to  the 
administration  of  mercury  than  do  the  corresponding  early  lesions  of 
the  long  bones. 

2.  Treatment  of  Recurrences  of  Hereditary  Syphilis  During 
Early  Childhood. — The  use  of  inunctions  is  here  tlie  treatment  pre- 
ferred. This  should  be  continued  at  least  till  all  symptoms  have 
disappeared  no  matter  whether  the  syphilitic  manifestations  are  of  a 
gummatous  of  or  a  condylomatous  nature.  It  is  a  good  plan  not  to  stop 
the  inunctions  at  once  after  cessation  of  all  symptoms  but  to  follow 
them  bj^  a  course  of  internal  administration  of  the  protiodide,  gi\ang  2 
eg.  (J  gr.)  a  day  for  two  or  three  weeks.  On  account  of  the  greater 
tendency  to  condylomatous  and  ulcerative  affections  of  the  mouth  and 
pharynx,  local  treatment  with  such  caustics  as  silver  nitrate,  bichlor- 
ide of  mercury,  etc.,  is  commonly  needed.  In  affections  of  the  central 
nervous  system  it  is  advisable  to  administer,  simultaneously  ■with  the 
inunctions,  sodium  iodide  internally  0.2-0.5  Gm.  {3-7J  gr.)  per  day 
and  to  keep  up  the  latter  for  from  four  to  six  weeks  after  cessation  of 
all  nervous  symptoms.  Isolated  swellings  of  certain  parts  of  bones  call 
for  the  application  of  mercurial  plaster  in  addition  to  the  combined  use  of 
iodides  and  mercury. 

3.  Treatment  of  Late  Hereditary  Syphilis. — Here  tlae  iodides 
take  first  rank  and  nearly  always  bring  about  rapid  improvement  if 
only  given  in  sufficiently  large  doses.  The  daily  dose  of  the  iodide  salt, 
potassium  or  sodium  iodide,  is  so  regulated  that  the  cliild  receives  a 
decigram  (IJ  gr.)  per  day  for  each  year  of  its  life.  The  iodide  is  pre- 
scribed in  aqueous  solution  without  syrup,  and  the  appropriate  dose  is 
given  three  times  a  day  in  sweetened  milk.  Bardach  recommends  iodfer- 
ratose.  In  case  of  severe  \'isceral  affections  and  in  ulcerations,  the  iodide 
alone  is  sometimes  inadequate  and  needs  to  be  supported  by  a  simul- 
taneous treatment  with  inunctions  of  2  to  3  Gm.  (30-45  gr.),  dailj'  of 
mercurial  ointment. 

Marfan  recommends  here  the  subcutaneous  injection  of  5  c.c.  of  a 
10  per  cent,  solution  of  mercuric  cyanide  every  second  day  in  combina- 
tion with  the  internal  treatment  with  iodide  of  potassium.  In  older 
children  intragluteal  injections  of  other  soluble  salts  of  mercury  can 
be  used,  especially  if  it  is  desirable  to  get  a  rapid  therapeutic  action  on 
account  of  the  involvement  of  some  important  organ  such  as  the  eye  or  ear. 


SYPHILIS  567 

In  general  there  is  no  difference  between  the  treatment  of  late 
hereditary  syphilis  and  that  of  the  tertiary  stage  of  accjuired  syphiUs. 
It  is  even  more  important,  however,  in  the  former  to  look  carefully 
after  the  general  health  and  strength  of  these  children  who  are  nearly 
always  cachectic.  Followng  the  specific  treatment  the  use  of  remedies 
containing  arsenic  is  to  be  recommended,  such  as  Fowler's  solution, 
Roncegno,  Levigo  or  Guber  water.  If  circumstances  permit,  drinking 
and  bathing  in  the  water  of  springs  that  contain  iodide  and  arsenic  are 
to  be  recommended. 

4.  Treatment  of  Acquired  Syphilis  in  Childhood. — This  is  in 
every  respect  the  same  as  in  the  adult;  especially  is  this  true  with  refer- 
ence to  the  treatment  of  the  primary  lesion  and  the  condylomata.  The 
constitutional  treatment  should  not  be  begun  until  the  secondary  man- 
ifestations have  set  in;  then  it  should  be  pushed  energetically  and 
whenever  possible  in  the  form  of  inunctions.  In  older  children,  the 
mouth  requires  exactly  the  same  care  as  that  of  adults  who  are  taking 
mercury.  I  do  not  think  it  necessary  to  carry  out  the  prolonged  inter- 
mittent treatment  in  the  acquired  syphihs  of  cliildhood  that  is  so  much 
favored  at  the  present  time  in  the  treatment  of  the  adult,  because  of 
the  relatively  easy  course  and  mild  character  of  contact  syphihs  in  child- 
hood. I  recommend  treatment  by  inunctions  until  all  secondary  symp- 
toms have  disappeared,  tliis  treatment  to  be  repeated  as  often  as  there 
recur  any  manifestations  of  the  disease.  One  should  make  it  a  rule, 
to  treat  all  recurrences  of  syphilis  on  the  skin  and  mucous  membranes, 
not  merely  locally,  but  also  constitutionally,  that  is,  with  mercury  in 
case  of  condylomata,  and  with  iodides  in  case  of  gummata. 


TUBERCULOSIS 

BY 

Professor  A.  SCHLOSSMANN,  of  Dusseldorp 

TRANSLATED    BY 

Dr.  ALFRED  F.  HESS,  New  York,  N.  Y. 


L  GENERAL  OUTLINE 
Definition. — Tuberculosis  is  an  infectious  disease  wliich  runs 
an  acute,  subacute,  or  even  a  markedly  chronic  course.  Occasionally 
death  supervenes  merely  days  or  weeks  after  infection,  accompanied 
by  astoundingly  severe  manifestations;  in  other  eases  the  disease  drags 
along  for  months,  years,  or  even  decades,  and  finally,  after  long  and 
tedious  sufTering,  results  in  a  general  disintegration;  in  yet  other  cases, 
the  human  organism  may  conquer  and  efTect  a  cure.  Tuberculosis  may 
be  locaHzed  in  the  various  organs,  or  may  be  rapidly  diffused  through- 
out the  body.  Its  clinical  pictures  are  manifold,  its  anatomical  aspects 
varying,  its  portals  of  entry  diverse.  The  common  factor,  which  unites 
all  these  different  manifestations  which  we  group  under  the  general 
title  of  tuberculosis,  is  the  causative  agent:  the  tubercle  bacillus.  The 
definition  of  tuberculosis  is  therefore  an  etiological  one,  in  contradis- 
tinction to  the  anatomical  significance  of  the  word  tubercle,  namely  a 
nodule.  It  includes  all  those  changes  and  conditions  which  are  caused 
by  the  tubercle  bacillus. 

Etiology — In  1882  Robert  Koch  discovered  the  tubercle  bacillus 
to  be  the  cause  of  tuberculosis,  and  thus  segregated  it  as  a  specific  dis- 
ease, and  enabled  the  varying  chnical  pictures  (miliary  tuberculosis, 
gelatinous  tuberculous  pneumonia,  tabes  mesenterica,  acute  hydro- 
cephalus, etc.)  to  be  united  into  the  etiological  group  of  tuberculosis. 
The  infectiousness  of  tuberculous  processes  was  previously  known 
(Villemin,  Cohnheim,  and  others)  although  disputed  over  and  over. 
Koch's  article  dispelled  all  doubts.  The  tubercle  bacillus  is  a  rod  1.5- 
4/*  long,  0.2-0.4/i  broad,  sHghtly  curved,  classified  by  Lehmann  as  a 
mycobacterium  under  the  hyphomycetes.  It  grows  slowly  when  cul- 
tivated, is  immobile,  and  possesses  a  pecuhar  characteristic  in  being 
stained.  It  cannot  be  decolorized  by  acids  after  having  been  deeply 
stained  by  means  of  a  dye  and  a  mordant.  Following  such  a  procedure 
(see  page  586)  it  appears  coarsely  granular  (Fig.  137).  Sunlight  kills 
the  bacillus  quickly;  intense  diffuse  light  less  quickly. 

Whereas  Koch  originally  considered  the  human  and  bovine  bacil- 

568 


TUBERCULOSIS  569 

lus  as  identical,  in  1901  he  changed  tliis  view  and  declared  them  to 
be  different.  First,  because  animal  experimentation  showed  that 
cattle  injected  or  fed  with  pure  cultures  of  human  tubercle  do  not 
develop  general  tuberculosis;  secondly,  because  man,  in  spite  of  his 
consumption  of  the  milk  or  flesh  of  tuberculous  animals,  rarely  de- 
velops primary  intestinal  tuberculosis.  The  investigations  of  P.  and 
E.  Biedert  and  of  Ganghofner  coincide  with  this  new  view  of  Koch's. 
[In  1896  Theobald  Smith  showed  the  non-identity  of  the  human  and 
bovine  tubercle  bacillus.]  They  showed  that  the  morbidity  of  tuber- 
culosis in  man  and  in  cattle  by  no  means  run  parallel,  that  on  the 
contrary,  where  tuberculosis  is  widespread  among  cattle  the  mortality 
of  this  disease  is  comparatively  low  and  \Tce  versa.  Koch's  view  as 
to  the  difference  of  species  between  the  human  and  bovine  bacillus 
has  been  actively  denied  by  other  authors  (Johne,  Arloing,  and  others). 
However  at  the  present  time  the  discussion  revolves  more  about  the 
point  as  to  whether  we  are  dealing  with  two  species,  different  and  totally 
separate,  or  with  one  species  descended  from  a  common  ancestor,  which 
has  gradually  accommodated  itself  to  its  host  and  to  its  particular 
parasitic  life,  and  thus  in  the  course  of  time  has  developed  two  different 
varieties  (Baumgarten). 

The  Imperial  Health  Department  in  an  article  by  Kossel  and  Weber 
has  made  the  following  pronouncement: — Among  the  bacilli  of  mam- 
malian tuberculosis,  two  types  may  be  distinguished,  wliich  are  best 
designated  as  the  human  and  the  bovine  types.  These  two  types  show 
characteristic  differences  as  regards  their  morphology,  cultural  aspects, 
and  virulence  for  rabbits  and  cattle.  A  metamorphosis  of  the  human 
type  into  the  bovine  type  as  the  result  of  experiments  on  rabbits,  goats, 
and  cattle  was  not  observed.  By  means  of  the  bacillus  of  the  human 
type  a  progressive  tuberculosis  in  cattle  could  not  be  produced.  In 
tuberculous  individuals  the  bovine  type  is  found  relatively  seldom, 
and  as  yet  only  in  children  under  eight  years  of  age.  The  tuberculous 
individual  is  a  source  of  danger  for  cattle  only  in  the  rare  instance 
of  his  excreting  bacilli  of  the  bovine  type.  On  the  other  hand,  the 
human  organism  seems  to  be  capable  of  receiving  the  bo\-ine  type  of 
bacillus.  Therefore  the  consumption  of  meat  of  tuberculous  animals 
containing  living  bacilli  of  the  bovine  type,  cannot  be  looked  upon  as 
insignificant  for  man.     Especially  is  this  true  during  childhood. 

The  tubercle  bacillus — we  speak  of  the  human  type  in  particu- 
lar— is  very  widely  distributed,  although  not  so  nearly  ubiquitous  as 
formerly  considered.  Wherever  the  tuberculous  indi\Tdual  goes,  pro- 
vided liis  excretions  and  secretions  contain  bacilli,  there  exists  the 
possibility  of  dissemination.  The  vehicle  of  infection  exists  not  alone 
in  the  sputum,  but  also  in  the  finest  particles  of  moisture  expelled 
in  the  act  of  speaking,  sneezing,  etc.     These  become  dr}',  and  are  later 


570  THE   DISEASES   OF   CHILDREN 

raised  with  the  dust.  In  the  open  air  this  is  of  little  consequence, 
as  the  sun  and  even  diffused  daylight  serve  as  most  excellent  disin- 
fectants. It  is,  however,  in  the  rooms  and  in  the  thickly  populated 
dwelhngs  of  the  poor,  where  sunhght  and  air  do  not  enter  or  find  their 
way  but  meagerly,  that  opportunity  is  offered  for  the  increase  of  the 
masses  of  virulent  bacilli.  The  bacillus  of  the  bovine  type  is  found 
more  especially  in  the  cow  stalls,  in  all  the  excretions  and  secretions  of 
tuberculous  animals,  as  well  as  in  their  flesh  and  blood.  It  is  found 
particularly  in  the  milk  of  tuberculous  cows,  even  in  those  not  suffering 
from  tuberculosis  of  the  udder,  and  in  the  products  of  raw  milk,  such  as 
cream,  butter,  cheese,  curds,  etc.  Thus  we  see  that  all  of  us  are  brought 
into  close  relation  with  the  bacillus  and  are  in  this  manner  exposed  to 
infection. 

Pathogenesis — Wc  must  mention  the  paths,  especially  in  child- 
hood, by  which  the  tubercle  bacillus  is  able  to  enter  the  human  body 
and  produce  tuberculosis.  This  question  is  by  no  means  settled,  but  we 
shall  attempt  to  discuss  the  contradictory  views  impartially,  and  to 
give  the  pros  and  cons  of  all  sides.  But  let  it  be  understood  from  the 
outset  that  no  one  claims  that  there  is  but  one  mode  of  infection  by 
tuberculosis. 

Innumerable  opportuuities  for  entrance  are  offered  the  tubercle 
bacillus,  and  at  one  time  or  another  it  may  gain  admittance  any- 
where in  the  body.  Indeed  the  bacilli  may  even  pass  through  the  intact 
skin,  if  they  are  briskly  rubbed  over  it;  far  easier,  however,  does  infec- 
tion follow  if  there  is  an  injury  to  the  cutis,  whether  it  is  caused  by  a 
tuberculous  object,  or  whether  the  infection  is  added  later.  Marked 
examples  of  this  class  of  cases  are  the  local  lesions  that  follow  the  hand- 
ling of  cadavers.  I  observed  a  case  where  a  tuberculous  mother,  fearing 
tuberculosis  following  vaccination  of  her  child,  sucked  the  wound,  and 
thus  produced  a  localized  tuberculosis  of  the  skin.  In  short,  the  tu- 
bercle bacillus  may  enter  the  human  body  at  any  point.  For  us,  however, 
the  important  questions  are:  Where  does  it  enter  in  the  majority  of  the 
cases?  Where  are  the  loci  minoris  resistentise?  Where  must  we  in 
general  expect  tuberculous  infection? 

There  are  three  different  views  upon  this  subject,  each  of  which 
explains  the  most  frequent  mode  of  infection  in  a  different  way: — (1) 
by  heredity  of  tuberculosis  (prenatal  infection) ;  (2)  infection  through  the 
air-passages  (aerogenic  infection);  (3)  infection  through  the  digestive 
tract  (enterogenic,  or  better,  alimentary  infection). 

1.     THE  HEREDITY  OF  TUBERCULOSIS 

Direct  heredity  of  tuberculosis  may  be  explained  in  two  ways: 
Either  the  disease  may  be  present  in  its  true  form  at  birth  or  in  the 
foetus  (congenital  tuberculosis),  or  the  bacilli  may  be  carried  from  the 


TUBERCULOSIS  571 

parents  to  the  offspring  and  remain  temporarily  latent,  only  to  show 
their  bhghting  influence  when  an  opjjortunity  presents  itself  (hereditary 
tubercidosis).  In  either  case  there  is  a  real  transmission  of  the  bacilli 
in  one  form  or  another,  and  not  merely  the  inheritance  of  a  predispo- 
sition, a  certain  increased  susceptibility  to  tuberculosis,  which  sooner 
or  later  may  lead  to  disease.  We  shall  later  return  to  the  question  of 
predisposition. 

The  possibihty  of  infection  of  the  foetus  with  tuberculosis  is  quite 
certain.  There  are  a  number  of  cases  both  in  human  beings  and  in 
animals  wliich  are  unquestionable  and  therefore  establish  the  fact 
without    doubt. 

The  number  of  such  cases  considered  reliable  and  unimpeachable 
varies  according  to  the  degree  of  scepticism  mth  which  one  approaches 
the  subject. 

About  twenty  cases  in  man  and  some  hundreds  in  cattle  are  sub- 
stantiated in  every  particular.  Huss  accepts  40  human  and  about  100 
bovine  cases.  Thieme  examined  the  foetuses  of  86  tuberculous  cows 
and  found  2  tubercular.  In  one  case  the  placenta  was  also  examined 
and  showed  tuberculous  changes.  Klepp  found  among  4068  newborn 
calves  0.64  per  cent,  suffering  from  congenital  tuberculosis,  and  in 
another  series,  among  847  calves,  1.18  per  cent.  Trustworthy  reports 
of  congenital  tuberculosis  in  calves  are  frequently  recorded;  among' 
such  are  those  of  Johne  in  1885,  Nocard  in  1896,  etc. 

As  a  type  of  a  convincing  case  in  human  pathology  I  shall  cite 
that  of  Lehmann: 

Woman,  40  years  of  age,  in  the  ninth  month  of  pregnancy,  having 
suffered  from  tuberculosis  for  a  long  period,  died  three  days  after  de- 
livery. Autopsy  showed  old  pulmonary  tuberculosis,  mihary  tubercles 
throughout  both  lungs,  scattered  omental  tubercles,  tuberculous  men- 
ingitis. Peritoneum  and  uterus,  including  placental  surface,  free  from 
macroscopic  tuberculosis.  The  child  died  24  hours  after  birth.  Au- 
topsy showed  aortic  lymph-nodes  the  size  of  a  pea  or  bean,  hard,  yellow, 
cut  surface,  granular.  The  bronchial  lymph-nodes  were  still  larger  and 
appeared  as  a  mass  the  size  of  a  cherry,  with  cut  surface  as  above. 
The  lungs  contained  submiliary  nodules,  some  as  large  as  the  head  of 
a  pin.  Liver,  spleen,  and  kidneys  showed  miliary  tuberculosis.  The 
nodules  of  all  these  organs  proved  to  be  tubercles  of  varying  age,  con- 
taining numerous  bacilli.  The  age  of  the  tubercles,  according  to  the 
microscopic  picture  must  be  judged  to  have  been  over  a  week;  the 
lymph-node  tuberculosis  of  far  longer  standing. 

However,  although  we  must  recognize  the  possibility  of  heredi- 
tary tuberculosis  in  man,  it  must  be  looked  upon  as  verj^  rare.  Berend 
inoculated  with  tuberculin  four  children  of  tuberculous  mothers  and 
many    born    of   healthy  mothers,  but    did  not  obtain  a  positive   reac- 


572  THE    DISEASES   OF   CHILDREN 

tioii  in  a  single  case.  I  myself  attempted  to  diagnose  tuberculosis  in 
early  infancy  by  means  of  tuberculin,  but  in  more  than  200  cases  did 
not  meet  with  a  case  of  congenital  tuberculosis,  in  spite  of  the  fact 
that  I  gave  especial  attention  to  children  of  tuberculous  mothers,  in 
whom  the  clinical  diagnosis  had  been  substantiated  by  means  of  the 
tuberculin  test. 

The  question  of  hereditary  tuberculosis  is  far  more  difficult  to 
settle.  Not  that  there  can  be  any  doubt  that  it  does  exist,  but  because 
it  is  not  easy  to  estimate  the  importance  of  heredity  in  the  etiology  of 
the  disease.  Hereditary  tuberculosis,  i.e.,  the  passage  of  living  and  viru- 
lent tubercle  bacilli  from  the  mother  to  the  offspring  certainly  occurs. 

The  first  proof  of  the  passage  of  tubercle  bacilh  from  the  maternal 
blood  into  the  fa'tal  placenta  and  into  the  fcetal  liver  was  offered  by 
Birch-Hirschfeld  and  Schmorl.  They  performed  a  Ctesarean  section 
post  mortem  upon  a  woman  who  died  of  miliary  tuberculosis  and  re- 
moved the  child  who  had  died  just  previous  to  its  mother.  Since  then, 
many  cases  have  been  reported,  among  which  are  some  certainly  open 
to  question,  as  to  whether  hereditary  or  congenital  tuberculosis  existed. 
In  such  cases,  a  few  days  or  weeks  after  birth,  an  advanced  tubercu- 
losis was  found. 

Bugge's  case  may  be  considered  one  of  strictly  hereditary  tuber- 
culosis: A  woman,  39  years  old,  suffering  from  tuberculosis  for  two 
years,  died  four  houis  post  partum,  and  her  child  26  hours  later.  Tuber- 
cle bacilh  were  found  in  the  blood  of  the  child's  umbihcal  vein  and  in  a 
section  of  an  hejiatic  blood  vessel.  Three  guinea-pigs  were  inoculated 
with  the  blood  from  the  umbilical  vein,  and  a  bit  of  lung  and  of  hepatic 
tissue  were  inoculated  subcutaneously.  All  three  animals  died  of  inocu- 
lation tuberculosis;    no  tuberculous  lesions  were  found. 

If  we  are  to  consider  hereditary  tuberculosis  merely  as  a  rare 
phenomenon,  of  httle  importance  in  the  genesis  of  the  great  white 
plague,  then  we  must  give  attention  to  another  possibility.  Our  atten- 
tion must  be  directed  not  alone  to  those  few  cases  in  which  tubercu- 
losis has  been  engrafted  previous  to  birth,  and  which  develop  the  dis- 
ease in  the  first  weeks  or  months  of  hfe  but  we  must  presuppose  that 
the  development  of  tuberculosis  may  follow  after  a  longer  interval. 

According  to  this  view,  the  tubercle  bacillus  is  transmitted  far 
more  frequently  to  the  offspring,  but  hes  dormant  in  the  tissues  of  the 
child,  and  for  the  time  being  does  no  harm.  Through  one  circum- 
stance or  another,  such  as  an  unfavorable  mode  of  hfe,  symbiosis  ^\dth 
other  disease  germs,  as,  for  example,  with  that  of  measles,  a  stimulus 
and  impulse  is  given  to  the  development  of  the  bacillus,  and,  in  that 
way,  to  the  outbreak  of  tuberculosis. 

We  would  thus  have  to  distinguish  between  the  infection  of  heredi- 
tary origin,  and  the  evolution  of  the  disease,  which  developed  weeks, 


TUBERCULOSIS  573 

months,  years,  ovon  decades  later,  from  the  seed  sown  previous  to 
birth.  We  wouhl  have  to  attribute  to  the  tubercle  bacillus  the  prop- 
tery  of  latency,  the  property  of  being  able  to  exist  permanently  in  its 
usual  form,  or  we  nuist  say,  that  it  may  live  in  the  tissues,  capable 
of  development  l)ut  in  a  form  not  yet  known  to  us  (larval  stage, 
Baumgarten). 

As  is  well  known,  Behring,  the  exponent  of  the  idea  of  infection 
during  the  first  few  days  of  extra-uterine  hfe,  beUeves  in  the  Ionge\aty 
of  the  tubercle  bacillus.  If  this  be  our  standpoint,  if  we  accept  the  pos- 
sibihty  of  latency,  then  we  can  understand  that  the  teaching  of  the 
hereditary  character  of  tuberculo.sis  is  gaining  more  and  more  support. 
This  view,  following  the  discovery  of  the  tubercle  bacillus,  was  en- 
tirely lost  sight  of.  Baumgarten  alone  persisted  during  all  these  years 
in  his  view  that  the  bacillus  was  frequently  transmitted  to  the  offspring, 
and  can  witness  with  pleasure  the  gradual  spread  of  his  ideas  (Baum- 
garteu,  Jahresbericht,  1898,  vol.  xii,  p.  570).  The  entire  teaching  of 
the  heredity  of  tuberculosis  will  always  be  coupled  with  the  name  of 
Baumgarten.  If  his  view  as  to  the  larval  stage  of  the  bacillus  be 
correct,  then  we  need  not  wonder  at  the  negative  results  of  the  tuber- 
culin injection  in  the  case  of  infants. who  have  the  germ  of  tubercu- 
losis in  their  organism.  For  the  reaction  is  not  the  result  of  a  tuber- 
culous infection;  the  rise  in  temperature  depends  rather  upon  the 
vital  reaction  of  the  bacilU  as  the  result  of  the  injection  of  tuberculin. 

When  we  weigh  the  pros  and  cons  of  such  a  broad  interpretation 
of  the  heredity  of  tuberculosis,  we  must  consider  in  the  first  place 
the  experience  of  veterinarians,  who  show  that  calves  which  are  im- 
mediately removed  from  their  tuberculous  mothers  and  reared  away 
from  all  source  of  contagion,  remain  healtliy. 

Human  pathology  offers  analogous  observations.  My  own  ex- 
perience would  seem  to  point  in  the  same  direction.  Immediately 
after  birth,  children  were  separated  from  their  tuberculous  mothers 
and  have  so  far  remained  free  from  tuberculosis.  On  the  other  hand, 
I  know  of  three  cases  where  the  children  of  tuberculous  mothers  were 
hkewise  carefully  guarded  and  separated  from  other  tuberculous  infec- 
tion, but  in  spite  of  this,  and  although  tliey  did  not  react  to  tuber- 
culin in  the  first  weeks  of  life,  they  developed  tuberculo.sis  with  a  positive 
tubercuhn  reaction  later  in  hfe. 

A  classical  experiment  of  this  nature  was  reported  l)y  Epstein  at 
the  Meran  meeting  of  German  scientists.  A  child  was  separated  from 
its  tuberculous  mother  immediately  after  birth  and  reared  in  the  found- 
hng  asylum  with  other  children.  Notmthstanding  this,  it  died  of 
tu])erculosis  during  the  first  year  of  its  hfe.  On  the  other  hand,  the  same 
author  reported  instances  wliere  immediate  separation  of  the  mother 
and  the  child  was  followetl   liy  absence  of  tuberculosis  in  the  offspring. 


574  THE   DISEASES   OF   CHILDREN 

As  an  argument  against  the  heredity  of  tuberculosis,  Heller  re- 
ports an  observation  concerning  some  guinea-pigs  iat  the  pathological 
institute  in  Kiel. 

The  pigs  are  descended  from  a  number  of  animals  which  were 
rendered  tuberculous  with  bovine  material  14  years  ago.  All  the 
descendants,  some  thousands,  have  always  been  healthy  and  strong  with 
the  exception  of  two  short  periods,  during  which  several  animals  died  of 
tuberculosis.  Heller  believes  this  infection  to  have  been  due  to  poor 
hay,  without  however  offering  any  proof  of  this  fact.  Considering  the 
great  rarity  of  spontaneous  tuberculosis  among  guinea-pigs,  we  may 
interpret  these  observations  in  another  way:  the  latent  disease  may 
have  been  transmitted  to  the  descendants,  only  to  become  active  at  the 
advent  of  some  exciting  factor,  such  as  poor  fodder.  Such  analogies 
may  readily  be  drawn  from  families  descended  from  tuberculous 
ancestors  where  we  find  a  sudden  outbreak  of  tuberculosis  in  one  or 
more  members. 

Two  facts  favor  the  probability  that  hereditary  tuberculosis  is 
by  no  means  very  rare.  First,  experiments  which  show  how  easily 
we  can  artificially  infect  the  offspring  (Friedmann);  second,  the  recent 
observations  of  Schmorl  and  Geipel  as  to  the  relative  frequency  of 
placental  tuberculosis. 

Although  a  number  of  cases  of  tuberculosis — by  no  means  an 
insignificant  number — are  of  hereditary  origin,  nevertheless  heredity  is 
not  to  be  regarded  as  the  sole  nor  even  most  important  cause  of  the 
fearful  scourge.  Nor  is  there  an  iron  law  that  the  offspring  of  tuber- 
culous parents  or  of  a  tuberculous  mother  must  develop  tuberculosis. 
On  the  contrary,  we  see  that  one  or  another  cliild  is  spared,  whereas 
others  become  its  victims.  Such  selection  may  be  seen  also  in  the 
transference  of  the  disease  among  members  of  the  same  family.  We 
can  therefore  formulate  two  conclusions:  (1)  tuberculosis  may  be,  but 
is  not  necessarily,  hereditary ;  (2)  tuberculosis  may  be  inherited  but 
may  also  be  acquired.  As  regards  the  mode  of  hereditary  transmission, 
we  have  the  following  possibiUties: 

1.  Germinal  Transmission.  The  seed  carries  with  it  the  germ  of  the 
disease:    (a)  from  the  father;   (b)  from  the  mother. 

2.  Placental  transmission:  (a)  with  tuberculosis  of  the  placenta; 
(b)   without  tuberculosis  of  the  placenta. 

3.  Transmission  intra  partum. 

1.       GERMINAL    TR.\NSMISSION 

The  male  as  well  as  the  female  seed  may  be  the  source  of  heredity. 
As  regards  heredity  from  the  male,  the  experiments  of  Friedmann  have 
shed  much  light.  He  injected  a  solution  containing  tubercle  bacilli 
into    the   vaginae  of   guinea-pigs  immediately  after  coitus,  and  killed 


TLBEUCULOSIS  575 

the  pigs  within  a  week.  In  all  the  embryos  tubercle  bacilli  were  found, 
intracellular  and  lying  witliin  the  embryonal  cell  layer.  The  organs 
of  the  mothers  were  in  every  case  healthy;  no  tubercle  bacilU  were 
found  in  the  vaginal  or  uterine  mucous  membrane.  As  the  semen 
of  those  suffering  from  genital  tuberculosis  is  almost  always  infectious, 
and  as  we  may  find  tubercle  Ixacilli  in  the  semen,  even  in  the  case  of 
general  tuberculosis,  where  the  testicle  and  epididymis  are  not  involved, 
the  possibility  of  transmission  from  the  father  must  be  regarded  as 
definitely  estabhshed.  Practically,  it  is  of  no  importance  whether  the 
spermatozoon  is  diseased  or  whether  it  is  healthy  and  merely  carries 
a  tubercle  bacillus  with  it.  Probably,  it  must  be  healthy  in  order  that 
the  development  of  a  foetus  may  be  brought  about.  The  possibility 
of  germinal  transmission  from  the  mother  is  also  presented  when  we 
consider  that  ovarian  and  genital  tuberculosis  is  by  no  means  rare. 

2.    PLACENTAL   TRANSMISSION. 

AVe  have  already  considered  the  question  of  transmission  of  tuber- 
culosis where  there  is  specific  disease  of  the  placenta.  But  is  it  per- 
haps possible  that  bacilh  may  pass  through  the  placenta  without  caus- 
ing tuberculous  lesions;  at  least  there  are  experiments  which  would  tend 
to  prove  tills. 

3.    TRANSMISSION     INTRA    PARTUM 

This  is  of  no  practical  importance  on  account  of  the  rarity  of  tuber- 
culosis of  the  vagina  and  of  the  external  genitals. 

« 

APPARENT  TRANSMISSION   (TRANSMISSION  OF  THE  DISPOSITION) 

Heredity  of  tuberculosis  by  means  of  the  tubercle  bacillus  must  be 
definitely  distinguished  from  what  is  termed  the  predisposition  to 
tuberculosis.  By  the  latter  is  understood  a  condition  of  the  tissues 
which  favors  a  growth  of  the  tubercle  bacillus  independent  of  its  por- 
tal of  entry.  A  body  so  predisposed  may  be  Ukened  to  a  field  pre- 
pared for  the  growth  of  some  special  seed.  The  resistance  wliich  every 
healthy  body  offers  to  pathogenic  microorganisms  is,  in  such  cases, 
specifically  diminished  towards  the  tubercle  bacillus.  That  certain 
individuals  are  more  susceptible  to  tuberculosis  than  others  is  a  fact 
that  was  recognized  even  at  the  time  when  the  theorj-  of  contagion 
held  sole  sway.  Koch  recognizes  the  importance  of  predisposition. 
Besides  taking  into  consideration  a  strong  family  tendency  to  the 
disease,  he  beUeves  that  the  element  of  time  is  important;  that  the 
same  person  is  far  more  likel}'  to  acquire  tuberculosis  at  periods  when 
his  power  of  resistance  is  diminished.  Baumgarten,  on  the  other  hand, 
in  consequence  of  the  views  stated  above,  gives  httle  weight  to  indi- 
vidual predisposition.     He  believes  that  man  has  a  superlative  predis- 


576  THE    DISEASES   OF   CHILDREN 

position  to  tuberculosis,  and  that  therefore  tliis  is  not  capable  of  being 
increased  in  the  case  of  any  individual. 

It  has  also  been  urged  against  the  theory  of  predisposition  that 
members  of  tuberculous  famihes  are  more  exposed  to  infection,  and 
that  they  are  therefore  more  frequently  afflicted  with  tuberculosis. 
Moreover  the  offspring  of  such  families  without  a  doubt  show  a  dimin- 
ished resistance  to  all  harmful  influences;  the  children  of  sickly  and 
weakened  parents  are  below  the  average  in  development  and  vital 
power.  Epstein  has  pointed  out  the  low  average  weight  of  infants  of 
tuberculous  mothers.  I  beheve  that  we  can  speak  of  paratuberculous 
manifestations  in  such  children,  following  the  analogy  of  the  offspring 
of  luetic  parents.  The  offspring  of  tuberculous  parents,  without  really 
having  tuberculosis,  at  times  show  certain  outward  signs  of  malnu- 
trition. To  what  extent  we  are  deahng  with  specific  tuberculous  man- 
ifestations in  such  children  we  cannot  at  present  say.  Other  chronic 
and  wasting  di.seases,  especially  when  combined  with  poverty,  may 
produce  similar  symptoms  in  the  offspring.  On  the  other  hand,  we 
find  many  children  whose  development  is  excellent  in  spite  of  a  tuber- 
culous taint. 

2.  INFECTION  DURING  LIFE 

We  now  come  to  the  consideration  of  tuberculosis  acquired  during 
hfe  and  first  of  all  to  the  so-called  aerogenic  infection.  Until  lately 
the  opinion  that  the  tubercle  bacillus  attacks  the  child  through  the 
respiratory  tract  was  not  only  the  accepted,  but  almost  the  uncon- 
troverted  view.  In  pediatric  hterature  the  cause  of  tuberculosis  is 
always  given  as  due  to  inhalation  of  the  bacillus.  Even  for  the  infant 
this  was  supposed  to  hold  good. 

As  a  proof  of  tliis  behef  it  was  stated  that  in  children  the  lungs 
and  bronchial  glands  are  the  sites  of  most  frequent  invasion.  This 
argument  formerly  seemed  to  me  sufficient,  and  I  too  was  of  this  opinion. 
Nor  can  it  be  gainsaid  that  tuberculosis  may  be  acquired  through  the 
respiratory  tract.  For  instance,  if  a  mason  should  inhale  an  infected 
sphnter  of  stone,  he  might  develop  an  inhalation  tuberculosis.  Even 
in  this  case,  however,  we  should  rather  expect  a  primary  tuberculosis 
of  the  larynx  or  of  the  larger  bronchi. 

In  childhood  opportunity  for  inhalation  of  tubercle  bacilh  is  espe- 
cially afforded  when  the  children  live  among  tuberculous  people.  This 
condition  is  particularly  dangerous  when  the  child  is  able  to  crawl 
about  on  the  floor. 

But  from  the  clinical  standpoint  the  question  at  once  presents  itself 
why  we  so  rarely  find  primary  tuberculosis  of  the  larynx  or  of  the  larger 
bronclu,  conditions  almost  unknown  in  younger  children.  How  is  it  that 
the  inhaled  bacilh  do  not  lodge  in  these  organs  instead  of  penetrating 
to  the  remotest  parts  of  the  lungs  and  to  the  bronchial  lymph-nodes? 


PLATE  30. 


TUBERCULOSIS  577 

Such  scepticism  may  be  answered  by  quoting  the  large  number 
of  experiments  whereby  tuberculosis  of  the  lungs  was  produced  by 
inhaling  powdered  tuberculous  material.  These  experiments  always 
gave  positive  results.  But  the  possibility  that  the  bacilli  entered  the 
intestinal  tract  as  well  as  the  inspiratory  tract  must  be  considered, 
and  therefore  the  possibility  that  the  infection  was  enterogenic  in 
nature.  We  must  uphold  von  Behring  in  his  statement  that  such  proofs 
of  inhalation   tuberculosis   must   be   carefully  and   critically  re^':ewed. 

In  spite  of  the  fact  that  the  basis  for  the  theory  of  aerogenic  in- 
fection was  far  from  sound,  this  belief  gained  general  credence.  Beh- 
ring's  starthng  communication  in  1903  served  to  make  us  reconsider, 
and  again  brought  forward  the  question  of  the  enterogenic  origin  of  tuber- 
culosis. Behring  maintained  that  tuberculosis  of  the  lungs  attacks  the 
babe  in  its  cradle,  but  that  the  milk  wliich  the  infant  drinks  is  the 
chief  source  of  danger. 

Behring's  \'iews  must  not  be  interpreted  to  mean  that  infection 
is  due  solely  to  bovine  bacilh,  as  he  said  even  in  liis  address  at  Ca-ssel 
that  the  danger  lay  in  milk  wliich  contains  tubercle  bacilh,  irrespective 
as  to  whether  the  bacilli  are  of  human  or  bo\ine  origin.  In  a  later 
article  he  emphasized  tliis  point  and  enlarged  upon  it,  adding  that 
bacilh  which  are  transmitted  from  mouth  to  mouth  in  the  act  of  kiss- 
ing, or  are  inhaled  with  the  dust  of  infected  rooms,  are  all  swept  into  the 
intestinal  tract  by  the  milk. 

When  these  new  views  of  Behring's  were  made  known,  the  natural 
assumption  was  that  following  an  enterogenic  infection  we  should 
have  to  look  for  the  primary  focus  in  the  intestinal  tract  and  in  its 
regional  lymph-nodes.  But  in  direct  contradiction  to  tliis  theory,  all 
investigations  report  that  primary  intestinal  or  mesenteric  lymph- 
node  tuberculosis  is  an  exceptionally  rare  occurrence,  notwithstanding 
which  Heller  found  primary  tuberculosis  in  tliis  region  in  30.7  per 
cent,  of  liis  cases  (140  autopsies  on  patients  who  died  of  diphtheria  but 
showed  some  tuberculous  lesion). 

Three  possibiUties  have  been  considered  as  regards  the  mode  of 
intestinal  infection:  (1)  that  there  develops  a  primary  tuberculosis 
of  the  intestine  which  gives  rise  to  involvement  of  the  mesenteric  lymph- 
nodes;  (2)  that  the  intestine  becomes  the  seat  of  a  nontuberculous 
infection  and  thus  allows  the  passage  of  tubercle  bacilli  and  other 
microorganisms  through  its  walls;  (3)  that  healthy  intestines  allow  the 
passage  of  tubercle  bacilh,  more  especially  during  cliildhood.  Some 
facts  add  weight  to  this  \'iew. 

If  the  infection  follows  according  to  the  second  and  third  methods, 
the  primary  focus  is  in  the  mesenteric  lymph-nodes.  Tliis  focus  may 
be  very  small,  not  even  macroscopic,  and  nevertheless  form  the  start- 
ing point  of  a  severe  general  tubcrcuh'sis.     Indeed  I  have  seen  cases 

11—37 


578  THE    DISEASES   OF   CHILDREN 

where  the  microscopic  examination  or  even  animal  inoculation  of  the 
lympli-nodes  was  necessary  to  show  the  presence  of  tuberculosis. 

There  is  one  more  possibility  and  one  wliich  has  received  too  little 
attention,  namely,  that  the  bacilli  can  pass  not  only  the  intestine  but 
also  the  mesenteric  lymph-nodes  without  causing  any  lesions.  We 
must  remember  that  during  digestion  a  strong  current  flows  from  the 
lumen  of  the  bowel,  and  thence,  traversing  the  intestinal  wall,  enters 
and  passes  through  the  mesenteric  lymph-nodes.  It  docs  not  seem 
unlikely  that  the  tubercle  baciUi,  attaching  themselves  to  the  fat 
globules,  may  pursue  this  same  course  through  the  distended  lymph- 
spaces  and  obtain  an  entry  into  the  thoracic  duct,  thence  into  the 
venous  blood  stream  and  right  auricle.  From  here  they  are  transported 
to  the  capillaries  of  the  lungs  where  the  blood  stream  is  slower  and 
then  enter  the  lymphatics.  They  next  find  their  way  to  the  bronchial 
lymph-nodes,  which  become  the  primary  seat  of  the  disease.  When 
the  lymph-nodes  cannot  receive  any  more  foreign  material,  dissemi- 
nation talves  place  in  the  lungs.  Animal  experimentation  shows  that  the 
injection  of  tubercle  bacilli  into  the  jugular  vein  leads  to  a  tuberculosis 
of  the  bronchial  lymph-nodes  and  lungs.  Also,  that  after  giving  tuber- 
culous food,  the  capillaries  of    the    lungs   are    full    of    tubercle  bacilU. 

Two  points  must  be  especially  considered.  First  that  the  diges- 
tive apparatus  does  not  commence  at  the  stomach,  but  at  the  lips;  so 
that  at  any  point  of  the  intestinal  tract,  from  mouth  to  anus,  infection 
may  occur.  And  also  that  there  are  certain  points  which  are  favor- 
able portals  of  entry  for  the  bacilh.  In  young  children  the  mucous 
membrane  of  the  mouth  is  a  locus  minoris  resistentia;  (of  course  not 
on  account  of  dentition)  as  are  also  the  pharyngeal  and  faucial 
tonsils,  which  through  their  crypts  afford  an  excellent  resting  place  for 
the  baciUi.  Whereas  the  stomach  and  small  intestine  appear  rarely 
to  be  the  seats  of  attack  of  the  tubercle  bacillus,  the  large  intestine 
is  frequently  invaded.  We  may  add  that  all  bacterial  infections  attack 
this  section  of  the  intestine  more  readily  than  any  other. 

The  tubercle  bacillus  may  enter  any  part  of  the  intestinal  tract. 
In  fact,  under  certain  conditions  it  maj'  gain  admittance  to  any  other 
part  of  the  surface  of  the  body,  whether  it  be  covered  \\ith  mucous 
membrane  or  skin.  According  to  our  present  knowledge,  we  may  say 
that  any  part  of  the  human  body  may  at  some  time  serve  as  the  portal 
of  entry  for  the  tubercle  bacillus.  But  as  to  the  question  of  the  cliief 
mode  of  infection,  our  knowledge  is  by  no  means  precise.  Tliis  being 
the  case,  we  must  not  direct  our  efforts  of  prophylaxis  to  one  point 
alone  but  must  con.sider  the  manifold  ways  in  which  a  child  may  be 
exposed  to  tuberculo.sis. 

Pathological  Anatomy. — The  tubercle  bacillus  ha^-ing  gained  en- 
trance into  the  body  in  one  way  or  another,  three  possibiUties  present 


TUBERCULOSIS  579 

themselves.  The  organism  may  conquer  the  bacillus  and  thus  pre- 
vent the  develoijnicnt  of  disease.  This  happy  event  may  be  brought 
about  by  the  lessened  virulence  or  small  quantity  of  the  infecting 
microorganisms,  or  by  the  strong  protective  powers  of  the  child. 

Second,  the  bacilU  may  remain  in  the  tissues  without  causing 
disease  or  increasing  in  number.  After  a  long  period  such  bacilh, 
becoming  more  virulent  through  weakening  of  the  protective  agencies 
of  the  body,  or  through  a  favorable  symbiosis  with  some  other  germ, 
may  increase  in  number  and  give  rise  to  the  dread  disease. 

The  tliird  possibility  is  that  immediately,  or  soon  after  entry 
into  the  body,  the  bacilli  bring  about  the  anatomical  changes  consti- 
tuting a  primary  tuberculous  lesion. 

The  tubercle,  wliich  gives  its  name  to  the  disease,  is  a  circum- 
scribed inflammatory  growth.  It  may  be  so  small  as  scarcely  to  be 
perceived  with  the  naked  eye,  or  it  may  grow  to  the  size  of  a  millet 
seed  or  a  pea.  Its  color  is  grayish  yellow.  Histologically  it  consists 
of  closely  packed  epitheUal  cells,  among  which  may  be  found  giant 
cells,  especially  toward  its  centre.  The  tubercle  bacilli  are  either  extra- 
cellular or  intracellular.  Characteristic  of  the  tubercle  is  the  lack 
of  blood  vessels.  The  tubercle  is  not  caused  by  the  \'ital  acti\'ity  of 
the  bacillus,  as  dead  bacilU  or  even  foreign  bodies  may  give  rise  to 
it.  However,  the  further  changes  that  the  tubercle  undergoes  are 
due  to  the  acti^ity  of  the  bacillus.  They  are  the  cause  of  the  rapid 
central  necrosis,  the  degeneration  that  transforms  the  nodule  into  a 
cheesy  mass. 

Besides  the  power  of  the  tubercle  bacilli  to  form  tubercles  and  to 
cause  cheesy  degeneration,  they  are  able  to  act  as  a  true  agent  of 
inflammation  and  to  cause  exudative  processes.  It  is  true  that  tliisis 
exceptional,  unless  they  have  spread  from  their  primary  focus. 

The  spread  of  the  germs  may  take  place  in  many  ways.  In  the 
majority  of  cases  it  occurs  by  contiguity;  one  tubercle  forms  next  to 
the  other,  until  they  are  all  fused  into  a  cheesy  mass,  forming  the  con- 
glomerate or  solitary  tubercle.  As  an  example  of  spread  by  contiguity 
we  may  cite  tuberculosis  of  the  intima  of  a  vessel  wliich,  by  contact, 
may  form  a  tubercle  on  the  intima  of  the  opposite  side  (Fig.  136). 

The  second  method  of  diffusion  is  by  means  of  the  body  fluids, 
the  secretions  and  the  excretions.  For  example  the  urine  may  be 
the  cause  of  development  of  tuberculosis  of  the  bladder  in  a  case  of 
tuberculosis  of  the  kidney.  Or,  again,  one  lymph-node  may  infect 
its  neighbor,  the  bacilU  being  carried  \\'ith  the  lymph-stream.  Indeed, 
retrograde  infection  is  conceivable  in  such  a  case  if  a  node  is  so  diseased 
that  it  offers  a  liindrance  to  the  lymph-stream  and  causes  stasis.  In 
such  a  case,  the  bacilU  would  be  carried  against  the  lymph-current  to 
lymph-nodes  distally  situated. 


580 


THE   DISEASES   OF   CHILDREN 


The  third  way  in  which  tuberculosis  may  spread  is  by  means  of  the 
blood.  The  bacilli  may  enter  the  blood  in  different  ways:  (1)  the  germs 
may  at  once  enter  the  blood  from  the  lymph  and  cause  primary  tubercu- 
losis of  the  vessel  wall.    This  may  later  give  rise  to  a  general  mihary  infec- 


FiQ.  136. 


Rz 


Tuberculosis  of  the  intima  of  a  pulmonary  vein. — f,  tubercle;  Rz,  wandering  cells;  E,  elastica 
intima,  in  which  the  elastic  fibres  in  the  vicinity  of  the  tubercle  are  completely  destroyed;  (i  and  <«, 
tubercles  in  the  vein. 


Fig.  137. 


\ 


Tubercle  bacilli  in  the  tissue  of  the  luag. 


tion;  (2)  a  tubercle  in  the  neighborhood  of  the  vessel  may  undergo  degen- 
eration and  rupture  through  the  vessel  wall;  (3)  bacilli  may  find  their 
way  from  the  lymph-stream  into  the  thoracic  duct  and  thence  into  the 
venous  blood,  finding  their  way  into  the  lungs  and  arterial  circulation. 


TUBERCULOSIS 


581 


In  any  of  these  three  ways  a  general  infection  by  tuberculosis  may 
be  brought  about.  However,  in  the  case  of  tuberculosis  by  contiguity, 
and  very  often  when  the  disease  is  spread  by  the  excretions  or  secre- 
tions, it  may  remain  localized  to  a  single  organ  or  confined  to  a  system 
of  the  body. 

The  peculiarities  in  the  pathology  of  the  disease  wliich  childhood 
exhibits  are:  very  rapid  growth;  early  degeneration  of  the  tubercle; 
typical  formation  of  miliary  tubercles  with  giant  cells,  especially  a.sso- 
ciated  with  subacute  miliary  tulserculosis;  frequent  tuberculo.sis  of  the 
intima  of  the  vessels;  the  predominance  of  gland,  bone,  joint,  brain, 
and  meningeal  tuberculosis.  Finally,  and  this  is  especially  true  of  young 
cliildren,  the  frequency  of  generaUzation,  and  the  rarity  of  signs  of 
reaction  in  the  surrounding  tissues,  which  indicates  the  sHght  tendency 
towards  heahng. 

Frequency. — Tuberculosis  is  a  very  frequent  disease  of  childhood. 
The  importance  of  tliis  fact  becomes  more  evident  the  longer  one 
studies  the  subject  of  tuberculo,sis  in  cliildren.  However,  the  statistical 
basis  for  such  an  opinion  is  indeed  scanty  and  contradictory  in  many 
particulars.  In  Vienna,  Hamburger  and  Sluka  found  tuberculous  lesions 
in  40  per  cent,  of  the  cases,  agreeing  with  the  figures  of  Schmorl  in  Dres- 
den. The  following  table  which  they  give,  shows  the  frequency  of 
tuberculosis  in  relation  to  the  various  ages: 


Age. 

Number 
of  Autopsies. 

Non- 
TuberculouB. 

Tuberculous. 

Percentage 
of  Tuberculous. 

1 

1.54 

sx 

80 
29 
28 
22 

130             ;              21                           16 

.51                                 kT                                   J'> 

3  and  4  ...           

33 

47                           59 
17                             60 
IS                             64 
17                           77 

12 
10 
5 

7  10 

11  14 

Total 

401 

241 

->"« 

We  may  thus  see  that  it  is  by  no  means  rare  to  find  tuberculosis 
in  children,  and  that  the  nearer  we  approach  puberty  the  oftener  it 
is  met  with.  The  clinician  must  therefore  look  for  tuberculosis  in 
cliildren  of  any  age. 

Diagnosis. — The  diagnosis  may  be  so  evident  that  it  can  be  made 
at  a  glance,  or  it  may  be  attended  with  the  greatest  chfficulties 
requiring  the  aid  of  all  the  resources  at  our  command. 

The  history  may  give  us  an  important  clue.  In  every  case  we 
should  learn  whether  either  of  the  parents  or  grandparents  hail  died- 
of  tuberculosis  or  haemoptysis,  or  whether  numerous  members  of  the 
family  died  at  an  early  age.  Such  inquiry  may  prove  negative  and 
nevertheless  we  may  be  deaUng  with  a  case  of  tuberculosis.  It  may  even 
happen  that  tuberculosis  in  the  cliild  discloses  the  fact  that  the  par- 


582 


THE   DISEASES   OF   CHILDREN 


ents  were  afflicted  with  the  disease.  I  could  cite  numerous  cases  where 
after  finding  definite  signs  in  the  ofTspring,  a  careful  examination  for 
the  first  time  disclosed  tuberculosis  in  the  mother  and  father.  Fur- 
thermore we  should  enquire  whether  the  child  lias  come  in  contact 
with  any  one  suffering  from  tuberculosis,  even  though  it  has  been  for 


Fig.  138. 


Fig.  139. 


Advaiicei!  pulmonary  tuberculosis.  Enlarged 
glaodi^  in  the  ueck. 


Posterior  view  of  same  child,  showing  ex- 
treme emaciation. 


only  short  periods  (servants,  midwives,  teachers,  etc.).  Often  tuber- 
culosis is  contracted  from  hving  with  a  tuberculous  person  for  a  short 
time,  especially  in  the  case  of  a  cliild.  Wassermann  reports  a  case 
where  an  infant  contracted  a  fatal  infection  as  the  result  of  living  for 
only  eight  days  with  a  person  suffering  from  tuberculosis. 


TUBERCULOSIS 


583 


After  the  history  has  been  taken,  an  inspection  of  the  patient 
should  aid  us  in  the  diagnosis.  Not  infrequently,  especially  in  chil- 
dren near  puberty,  we  meet  with  the  typical  phthisical  habitus.  The 
peculiar  build  and  carriage  recognized  even  by  the  laity,  tells  us  that 
we  are  dealing  ■with  a  tuberculous  individual.  These  children  are  tall 
for  their  age,  or  at  least  appear  to  be,  on  account  of  the  disproportion 
between  the   width  of  their  chests  and  their  height.     The  long  and 


Fig.  140. 


Fig.  141. 


Pulmonarj' tuberculo.-is  in  a  thirteen-year-old  girl. 


The  same  case.    Raising  the  ann.s  shows  the 
"  phthisical  habitus  "  more  plainly. 


tilin  extremities  and  scrawny  necks  enhance  this  effect.  The  fingers, 
too,  are  long  and  thin,  the  distal  digits  being  thickened  and  club-shaped. 
The  chest  is  flat,  expanding  feebly  upon  deep  inspiration.  The  scap- 
ula? are  situated  low  and  the  shoulders  converge  anteriorly;  the  ribs 
stand  out  prominently  (Figs.  138-141). 

Although  the  picture  is  shocking  when  it  presents  itself  in  its 
most  extreme  form,  it  may  be  even  attractive  in  the  case  of  certain 
individuals.     Artists  have  frequently  pictured  a  beautiful  type  of  con- 


584  THE   DISEASES   OF   CHILDREN 

sumptive.  Only  recently,  I  saw  a  little  girl  (Plate  30)  whose  long  eye- 
lashes, bright  eyes,  wealth  of  hair,  and  sweet  expression  made  her 
a  picture  of  loveUness.  The  same  plate  shows  the  wasted  figure  of 
the  child  when  seen  unclothed,  and  gives  a  schematic  drawing  of  the 
extent  of  the  lesions  which  were  found  in  the  lungs.  Children  under 
ten  years  of  age  are  more  rarely  of  this  type.  But  even  very  young 
persons  may  have  the  phtliisical  habitus.  In  them  it  generally  pre- 
sents itself  as  extreme  emaciation.  Further  inspection  may  reveal 
bone  or  joint  diseases,  perhaps  of  the  knee  or  hip,  or  we  may  notice 
a  spina  ventosa  or  involvement  of  the  superficial  lymph-nodes,  espe- 
cially of  those  cervical  nodes  which  lie  close  to  the  lower  jaw  or  in  the 
supraclavicular  region.  Scars  in  tliis  region  may  tell  of  lymph-nodes 
which  have  ruptured  in  this  area. 

The  skin  is  often  grajdsh  yellow,  discolored,  and  of  striking 
dryness.  Tuberculids,  lupus,  or  peculiar  furuncular  lesions,  which 
remain  in  a  stationary  state  for  weeks  or  months,  or  show  no  tendency 
to  heal,  have  of  late  been  frequently  described  as  concomitant  syftip- 
toms  of  a  general  tuberculosis.  In  young  children  a  pecuUar  odor, 
the  cause  of  which  is  not  yet  known,  is  frequently  noticeable. 

Palpation  will  disclose  the  lymph-nodes  just  mentioned.  Their 
lack  of  tenderness  speaks  against  simple  inflammatory  origin.  The 
spleen  is  often  much  enlarged,  especially  in  general  tuberculosis, 
although  it  cannot  be  considered  a  pathological  symptom. 

The  fever  is  in  no  way  typical.  It  is  rather  dependent  upon 
infection  due  to  secondary  microorganisms.  There  are  indeed  cases  of 
tuberculosis  in  children  which  give  rise  to  httle  or  no  fever. 

Auscultation  and  percussion  are  of  great  diagnostic  value  in  those 
cases  of  chronic  illness  where  an  infiltration  of  the  lungs  is  present. 
In  cliildhood,  however,  we  do  not  find  tuberculous  infiltrations  or 
tuberculous  pneumonias  which  develop  gradually  and  progress  slowly. 

Physical  signs  enable  us  to  make  the  diagnosis  of  tuberculosis 
only  when  we  find  a  cavity.  Ha-moptysis,  provided  we  are  sure  of  its 
pulmonary  origin,  also  allows  of  this  diagnosis.  The  respiration  in 
tuberculosis  is  by  no  means  characteristic.  The  cough  may  at  times 
be  suggestive,  for  example,  paroxysmal  attacks  point  to  tuberculosis 
of  the  bronchial  lymph-nodes.  It  may,  however,  resemble  in  every 
respect  the  cough  of  other  pulmonary  affections.  Examination  of  the 
urine  does  not  supply  any  definite  diagnostic  criteria.  Indicanuria 
points  to  tuberculosis,  but  its  absence  is  not  positive  evidence  against 
the  presence  of  the  disease.  Convulsions,  which  in  miliary  tubercu- 
losis usher  in  the  end,  and  those  focal  symptoms  which  originate  from 
solitary  tubercles  in  the  brain,  are  worthy  of  mention.  Ophthalmo- 
scopic examination  of  the  fundi,  in  exceptional  cases,  shows  the  pres- 
ence of  chorioid  tubercles  early  in  the  disease,  and  thus  clinches  the 


TUBERCULOSIS  585 

diagnosis.  [This  examination  is  not  infrequently  of  diagnostic  value 
in  the  differentiation  of  cerebrospinal  and  tuberculous  meningitis  and 
should  not  be  omitted  in  cases  where  the  etiology  is  not  clear. — 
A.  F.  H.] 

Whereas  the  methods  of  examination  wliich  we  have  thus  far  men- 
tioned afford  us  only  exceptionally  absolute  proof  of  the  nature  of 
the  disease,  we  must  now  consider  two  pathognomonic  tests:  first,  the 
finding  of  the  tubercle  bacillus,  and  second,  the  injection  of  tubercuhn. 

Finding  the  tubercle  liacillus  in  childhood  is  associated  with  far 
greater  difficulties  than  in  the  case  of  adults;  for,  whereas  the  latter 
generally  suffer  from  open  tuberculosis,  that  is,  from  a  form  of  the  dis- 
ease wliich  communicates  with  the  bronclii  and  upper  air-passages 
cliildren  more  often  are  affected  with  the  closed  variety.  Furthermore, 
babies  swallow  their  sputum.  However,  vnder  all  circumstances  ichere 
tuberculosis  is  suspected  ice  shoidd  try  to  find  the  bacillus.  If  no  sputum 
can  be  obtained  we  may  tickle  the  entrance  of  the  larynx  with  a  bit 
of  cotton  held  by  a  forceps,  in  order  in  that  way  to  excite  an  attack 
of  coughing  which  may  bring  up  some  mucus;  or  we  may  introduce 
a  catheter  into  the  larynx  and  aspirate  material  for  examination.  Some 
have  recommended  washing  out  the  stomach,  especially  early  in  the 
morning,  in  order  to  obtain  bacilU  wliich  may  have  been  swallowed 
in  the  course  of  the  night.  In  the  case  of  older  children  we  may  resort 
to  Blume's  method  of  having  the  child  cough  upon  a  glass  shde,  in  order 
to  obtain  a  few  drops  for  microscopical  examination. 

If  these  methods  fail,  an  examination  of  the  stools  for  tubercle 
baciUi  must  be  made;  and  if  they  are  found,  they  cannot  be  consid- 
ered due  to  an  intestinal  lesion.  They  may  equally  well  have  been 
swallowed  and  have  their  source  in  a  tuberculosis  of  the  tonsil  or 
of  the  lungs.  Strassburger's  sedimentation  method  is  used  to  detect 
the  baciUi.  This  consists  in  mixing  a  small  amount  of  stool  with  water, 
adding  alcohol  or  alcohol  and  ether  to  the  supernatant  fluid,  then 
allowing  it  to  settle  and  examining  the  sediment  for  bacilh. 

We  must  also  not  neglect  to  look  for  bacilU  in  the  sediment  of  the 
urine.  They  may  be  -found  not  only  where  tuberculosis  of  the  genito- 
urinary tract  exists  but  at  times  when  the  disease  is  located  elsewhere. 

Examination  for  tubercle  bacilli  in  sputum,  in  the  urinary  sediment, 
in  feces,  and  in  the  stomach  contents  is  best  carried  out  as  follows: 

1.  Spread  the  material  as  thin  as  possible,  allow  it  to  dry,  and 
fix  by  passing  through  the  flame  three  times. 

2.  Stain  for  two  minutes  with  carbolfuchsin,  holding  it  over  the 
flame  until  the  solution  begins  to  steam  [fuchsin  1  Gm.  dissolved  in 
10  c.c.  of  absolute  alcohol,  to  which  100  c.c.  of  carbohc  acid  (5  per  cent.) 
is  added]. 

3.  Wash  with  water. 


586  THE    DISEASES    OF   CHILDREN 

4.  Decolorize  for  about  30  seconds  in  a  hydrochloric  acid-alco- 
hol mixture  (10  c.c.  cone,  hydrochloric  acid,  990  c.c.  70  per  cent,  alcohol). 

5.  Wash  with  60  per  cent,  alcohol  until  all  the  red  color  is 
removed. 

6.  Counterstain  with  weak  aqueous  methylene  blue  solution  for 
1-2  minutes  (1  c.c.  cone,  aqueous  sol.  of  methylene  blue  to  10  c.c.  of 
distilled  water). 

7.  Wash  and  dry  with  filter  paper. 

If  the  excreta  show  no  tubercle  bacilli,  we  may  resort  to  an 
examination  of  the  blood.  For  this  purpose,  1-2  c.c.  of  blood  are 
obtained  from  the  median  vein  and  injected  either  intraperitoneally 
or,  if  we  are  anxious  to  know  the  result  more  quickly,  into  the  mam- 
mary gland  of  a  guinea-pig  which  is  suckling  its  3'oung.  How'ever, 
even  if  the  blood  contains  bacilli,  we  shall  probably  not  discover  the 
fact  until  the  patient  has  died.  Nevertheless  the  method  is  of  value 
to  establish  the  diagnosis  when  we  do  not  believe  an  autopsy  will 
be  granted. 

Lumbar  puncture  maj^  show  the  bacilh  in  miliary  tuberculosis  in 
spite  of  the  absence  of  all  clinical  symptoms  which  might  point  to 
an  involvement  of  the  meninges. 

If  the  find  the  bacillus  in  one  or  other  of  the  above  ways,  the  diag- 
nosis is  established.  How-ever,  if  we  do  not  find  it,  as  is  so  often  the 
case  in  the  early  stages,  we  may  resort  to  the  second  method,  which 
consists  in  the  Diagnostic  Injection  of  Tuberculin. 

It  is  best  to  u.se  Koch's  old  tuberculin  diluted  with  water  without 
the  addition  of  any  antiseptic.  For  convenience  a  1:100  sterile  solu- 
tion may  be  kept  on  hand  and  diluted  to  the  proper  strength  when 
used.  The  stock  solution  should  be  kept  in  a  dark,  cool  place  and 
should  not  be  used  if  it  has  become  cloudy.  An  ordinary  hypoder- 
matic syringe  may  be  employed  and  the  injection  given  subcutane- 
ously  in  the  back  or  abdomen,  the  skin  having  previously  been 
disinfected. 

We  cannot  resort  to  the  tuberculin  test  unless  the  patient  has 
been  free  from  fever  for  a  time.  I  have  the  temperature  taken  for 
a  48  hour  period,  and  if  it  rises  above  99.5°  F.  I  do  not  use  tuberculin. 

The  strength  of  the  injection  is  regulated  according  to  the  condi- 
tion that  we  find.  We  should  never  begin  with  a  larger  dose  than 
0.001  Gm.  and  in  general  it  is  better  to  use  i-tV  of  tliis  amount,  namely, 
0.0001  Gm.  Tliis  is  especially  true  of  the  cases  where  we  find  an 
infiltration  of  the  lungs. 

From  4  to  6  hours  after  the  injection  the  temperature  begins  to  rise, 
at  first  gradually  and  then  in  2  to  4  hours  precipitously.  It  then  gradu- 
ally falls  and  reaches  normal  before  the  24  hours  are  past  (Fig.  142). 
In  other  cases  it  remains  liigh  for  24  hours  or  even  longer  (Fig.  143). 


TUBERCULOSIS 


587 


This  protracted  curve  is  supposed  to  be  more  frequent  in  children  than 
in  adults.  In  a  third  group  of  cases,  the  temperature  does  not  rise  until 
later  (Figs.  144  and  14.5)  or  the  rise  occurs  very  gradually  (Fig.  146). 
A  reaction  may  be  considered  positive  if  the  rectal  temperature 
rises  above  37.6°  C.  (99.7°  F.)  or  if  it  rises  0.5°  C.  (0.9°  F.)  higher  than 

Fio.  142. 


H  i 


VP^ 


Tuberculiu  ri-acliuii  with  rapni  ri^e  aiij   rapij  fall. 


Fig.  143. 


ff-    -  '  '  \  '-^tI^^^p^— ^ 


3; 


:^ 


H^ 


m 


IlliillilHiiilllilliliillliilllliiliilliiiillliillil 


Protracted  tuberculin  reaction. 


Fig.   144. 


444. 


,^-Ur 


Delaj'ed  tuberculin  reaction. 

it  did  in  the  4S  liours  precciHng  the  injection.  If  the  reaction  prove 
negative  or  doubtful,  the  injections  must  be  repeated  with  gradually 
increasing  doses,  until  0.01  Gm.  is  reached.  I  have  never  found  the  tuber- 
culin reaction  to  mislead  me.  In  all  cases  where  I  have  been  able  to 
follow  the  condition  of  the  child  or  to  perform  an  autopsy.  I  have 
found, the  tubercuhn  test  reliable.  Where  the  injection  has  proved 
negative,  we  have  never  found  tuberculosis.     Nor  have  we  ever  seen  any 


588 


THE   DISEASES   OF   CHILDREN 


harm  done  by  the  injection,  as  some  others  have  reported.  Of  course  the 
above  rules  must  be  strictly  adhered  to;  but  I  shall  return  to  tliis  later. 
We  must  not  forget  that  Escherich  has  called  attention  to  an  inflam- 
mation at  the  site  of  the  injection,  a  more  or  less  circumscribed  redness 
and  swelling.     Tliis  he  beheves  occurs  only  in  tuberculous  individuals. 

Fig.  145. 


*iunE 

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. 

u 

T 

r- 

^ 

~ 

^ 

~ 

„ 

i «, 

:   :   ,             ...■■■      .   i   :   1   1   M   l,Ui.,l !,| 

,        , 

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. 

.. 

,-.      c,     .-,■-■■      ■      .      f      .      ,1     ,; 

:      .     ,     .      :-.:..      1     ■>  „ 

.    , 

42* 
41' 
40* 
39' 
»• 
97  • 

as* 

to 
w 

i  ■           i         ■ 

!             M 

t" 

.;;■"■! 

-'  •-' 

MM     :     M 

!         .          :         ■ 

';.':''■.'■ 

i    ;   M   j 

:   .   ,   ,   , 

1  l-i  !  1 

-irij-j 

1   i   1-  i  p 

LM  1  ■ 

:  ^^M    ^  -  :  i 

r  1   i   j   t 

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- 

Oil 
M 
00 

j    •-H.--r-- 

~^~rT}'V 

1      i  ~T     '      : 

M    ■    ^    ;         ^    '    '    I    I 

' 

.  ;  :  ■  ; 

'     ;  ■  M  i 

L 

1 

-M 

Same  child  aa  in  Fig.  144.     Reinfection  showing  the  same  reaction  type  to  tuberculin. 

Fio.   146. 


1W1 


ISlvi    !♦  4 


i    t- 


^rrr 


irnw 


8  '0  li  ; 


I  I 


-\\ 


_L 


iXjL 


IIP 


NtUi 


Step-like  ascent  of  tuberculin  curve. 
Fio.  147. 


irregular  curve  in  a  case  of  miliary  tubprculosi".     Tiijecfion  given  a  week  previous 
to  death  substantiated  the  diagnosis. 

The  agglutination  reaction,  as  recommended  by  Arloing  and  Cour- 
mont,  has  proved  of  absolutely  no  value. 

Prophylaxis. — Tn  considering  measures  of  propln^laxis  we  shall  first 
speak  of  the  general  precautions  wliich  should  be  taken  to  protect  all  cliil- 
dren,  and  then  of  the  special  precautions  which  those  should  take  who  are 
predisposed  to  tuberculosis  either  by  heredity  or  by  their  surroundings. 


PLATE  31. 


TUBERCULOSIS  589 

1.  General  Precautions:  (a)  increasing  the  powers  of  resistance 
of  the  body,  (b)  care  that  the  cliild  be  subjected  to  the  contagion  of 
the  tubercle  bacillus  as  rarely  and  as  late  in  life  as  possible. 

(o)  As  regards  increasing  the  powers  of  resistance,  it  would  carrj' 
us  too  far  afield  to  repeat  what  has  already  been  said  in  the  general 
part  of  tills  work.  Of  prime  importance  in  this  connection  is  breast- 
feeding. A  child  nourished  at  the  breast  has  greater  resistance  against 
all  infections,  including  infection  by  the  tubercle  bacillus.  If  artificial 
feeding  be  unavoidable,  l)y  all  means,  let  it  be  carried  out  rationally, 
as  regards  quantity.  Overfeeding  as  well  as  underfeeding  during 
infancy  weakens  the  protective  agencies  of  the  body.  A  mixed  diet  in 
which  green  vegetables  have  their  proper  share,  must  be  begun  at  the 
proper  time. 

Alcohol  paves  the  way  for  tuberculosis  in  childhood  as  at  other 
periods  of  hfe.  It  should  be  absolutely  excluded,  forbidding  even  a 
glass  of  beer  or  medicinal  wine.  In  order  to  protect  the  child  against 
tuberculosis,  its  body  should  be  hardened,  its  muscles  made  firm  by 
exercise,  and  its  lungs  developed. 

Anaemia,  which  is  found  more  often  among  the  pampered  children 
who  idle  indoors,  must  be  treated  by  dietetic  measures.  Furthermore, 
great  attention  must  be  paid  from  early  infancy  to  disorders  of  the 
upper  respiratory  organs.  Any  catarrh  of  the  nose  or  throat  should 
receive  prompt  treatment;  enlarged  tonsils  and  adenoids  should  be 
removed  early. 

(b)  In  the  prevention  of  infection  a  good  general  rule  is  that  "a 
priori  every  person  is  suspected  of  having  tuberculosis."  Strangers 
should  not  be  allowed  to  fondle  cliildren,  especially  during  infancy,  and 
above  all,  kissing  should  be  prohibitetl.  Tuberculosis  should  be  borne 
in  mind  when  selecting  a  nurse.  Her  family  history  should  l^e  considered 
as  well  as  her  own  physical  condition,  and  if  there  is  any  doubt,  resort 
should  be  made  to  the  tuberculin  test.  The  choice  of  the  other  ser- 
vants is  of  equal  importance.  Any  one  suffering  from  an  active  tubercu- 
losis should  not  be  tolerated  in  the  vicmity  oj  children.  Cases  where  a 
phthisical  nurse  has  infected  a  child  of  healthy  parents  are  by  no  means 
unheard  of.  Midwives  ought  also  to  be  examined  for  tuberculosis. 
Later  in  hfe,  children  may  be  exposed  to  this  infection  by  their  teachers 
or  fellow  pupils.  Institutions  that  farm  cliildren  to  private  individuals 
should  see  to  it  that  there  is  no  danger  from  this  source. 

When  people  change  their  residence,  they  shouhl  always  con- 
sider the  possibility  that  the  previous  tenant  may  have  had  tubercu- 
losis, and  clean  or  disinfect  accordingly.  These  precautions  are  recom- 
mended for  summer  resorts  and  watering  places.  Such  objects  as  retain 
the  dust,  e.g.,  carpets,  upholstered  furniture,  should  be  exposed  to  the 
sun  before  they  are  used,  or  better  still,  they  should  be  entirely  dis- 


590  THE   DISEASES   OF   CHILDREN 

pensed  with  in  cliildrcii's  rooms.  Frequent  sunning  and  ventilation 
will  help  to  exterminate  any  tubercle  bacilli  that  may  be  present. 
Creeping  children  should  not  he  permitted  to  wander  about,  but  should 
be  confined  to  a  small  enclosed  part  of  the  room  wliich  has  been  covered 
with  a  clean  sheet. 

From  earliest  infancy  we  ought  to  combat  the  miscliievous  habit 
children  have  of  putting  everything  into  their  mouths  and  of  sucking 
their  fingers.  Nipples  or  "pacifiers"  are  a  cause  of  many  diseases, 
including  tuberculosis,  as  we  can  readily  understand  when  we  see  them 
taken  from  the  dirty  floor  and  put  into  the  child's  mouth.  Another 
habit  that  should  not  be  tolerated  is  the  tasting  of  the  milk  by  the 
mother  or  by  the  nurse,  either  by  putting  the  nipple  or  the  spoon  into 
her  mouth  before  giving  it  to  the  child. 

Children  should  be  kept  away  from  people  who  cough.  They  should 
be  taught  cleanly  habits,  such  as  washing  their  hands  before  eating, 
for  the  nails  are  a  favorite  site  for  the  deposit  of  dirt  and  bacteria. 

Although  human  beings  are  by  far  the  most  frequent  source  of 
contagion,  tuberculosis  of  cattle  must  be  taken  into  consideration. 

It  is  a  disgrace  to  be  compelled  to  admit  that  a  large  proportion 
of  our  milch  cows  have  tuberculosis,  and  that  their  products,  milk, 
cream,  butter,  meat,  etc.,  are  not  without  danger.  We  should  there- 
fore give  infants  only  such  milk  in  the  raw  state  as  comes  from 
carefully  inspected  cows  (frequently  repeated  tubercuUn  tests). 
Prophylaxis  against  tuberculosis  without  guarding  against  the  evils 
of  tuberculous  cattle  is  a  vain  endeavor. 

We  should  also  take  care  that  the  milk  of  healthy  cattle  is  not 
contaminated,  an  occurrence  wluch  is  by  no  means  rare.  Therefore 
no  one  suffering  from  tuberculosis  should  be  allowed  to  handle  the  milk 
at  any  stage. 

Recently  Behring  has  introduced  a  vaccine  which  promises  to  ac- 
complish the  immunization  of  cattle  against  tuberculosis.  For  man, 
however,  no  such  means  has  been  discovered. 

2.  Special  Prophylaxis  for  Children  Predisposed  to  Tuberculosis. — 
Every  tuberculous  individual  who  marries  and  has  a  family  assumes 
a  great  responsibiUt)^  No  person  whose  tuberculosis  is  not  positively 
healed  should  be  encouraged  to  marry.  A  child  born  of  tuberculous 
parents,  especially  if  the  mother  is  affected,  should  be  taken  away 
from  its  parents  immediately  if  we  wish  to  provide  for  its  future  wel- 
fare. However,  this  radical  measure  will  never  be  comphed  with  unless 
the  mother  is  in  an  advanced  stage  of  tuberculosis.  The  most  we  can 
accomplish  is  to  give  the  mother  warning  and  advice. 

How  shall  we  nourish  the  child?  Where  the  social  conditions 
are  good  in  every  particular,  I  recommend  the  employment  of  a  good 
wet-nurse.     However,  such  instances  are  the  exception  and  generally 


TUBERCULOSIS  591 

the  child  must  be  fed  with  cow's  milk.  Only  when  the  mother's  tuhcr- 
culosis  is  incipient  can  we  risk  an  attempt  at  maternal  nursing. 

From  the  standpoint  of  the  tuberculous  mother,  it  is  far  more 
injurious  for  her  to  become  pregnant  again  after  a  short  interval  than 
it  is  for  her  to  nurse. 

It  is  very  true  that  occasionally  tubercle  bacilh  have  been  found 
in  the  milk  of  tuberculous  women.  However  they  are  few  in  num- 
ber and  play  an  unimportant  part  when  compared  to  the  many  cases 
of  infection  of  the  child  by  the  tuberculous  mother  in  the  crowded 
quarters  of  the  poor.  This  is  especially  true  where  the  mother  prepares 
the  infant's  milk. 

If  the  tuberculous  parent  be  the  father,  then  the  child  should 
surely  be  nursed  by  the  mother.  Theoretically  the  father  should  be 
separated  from  the  child  but  in  practice  tliis  is  rarely  feasible.  It  is 
principally  among  the  poor  that  our  good  advice  is  difficult  to  follow. 
When  the  father  is  in  the  advanced  stages  of  the  disease  and  thus 
unable  to  support  the  family,  the  mother  is  compelled  to  become  the 
bread-winner.  The  father  in  his  turn  attends  to  the  household  and 
to  the  children,  and  becomes  a  dangerous  source  of  infection. 

Cliildren  who  have  been  subjected  to  tuberculosis  must  be  reared 
with  especial  care.  Every  catarrh  of  the  upper  respiratory  passages 
must  be  given  particular  attention,  and  infectious  diseases  such  as 
measles  and  whooping-cough  must  be  carefully  guarded  against.  Men- 
tal and  physical  strain  should  be  avoided.  Nourishment  and  physical 
development  are  very  important.  Under  some  conditions,  climatic 
measures  exert  a  good  prophylactic  influence.  In  the  .summer  such 
children  should  be  sent  to  the  sea-shore  for  a  period  of  at  least  six  or 
eight  weeks.  Such  outing  should  be  repeated  and  in  some  cases  pro- 
longed for  months  or  even  years.  Mountain  air  is  hkewise  beneficial. 
Tonics  are  also  of  value,  especially  arsenic  when  prescribed  in  the  form 
of  some  natural  mineral  water.  Iron  may  be  used,  preference  being 
given  to  non-alcohoUc  preparations.  At  times  a  "Mastkur"  or  forced 
feeding,  may  be  of  benefit. 

Finally  we  must  repeat  that  a  successful  campaign  against  tuber- 
culosis can  be  waged  only  if  ive  prevent  infection  during  childhood. 
For  it  is  during  this  period  of  life  in  the  great  majority  of  cases  that 
the  infection  takes  place,  although  the  disease  may  not  become  manifest 
until  later  in  life. 

Treatment. — As  yet  we  know  of  no  specific  treatment.  We  hope 
for  the  best  from  Behring's  latest  investigations,  especially  Ms  "tulase." 
Koch's  tubercuUn  has  a  curative  effect  in  some  cases,  but  it  is  not 
widely  apphcable,  and  is  valueless  in  generahzed  tuberculosis.  In 
spite  of  these  therapeutic  deficiencies  we  must  not  be  pessimistic,  for 
tuberculosis  is  in  certain  stages  a  curable  disease. 


592  THE   DISEASES   OP^   CHILDREN 

11.     SPECIAL  PART 

We  now  consider  the  different  clinical  manifestations  of  tubercu- 
losis in  childhood.     These  are  general  or  local. 

A.    GENERALIZED  TUBERCULOSIS 
Tliis   includes   the    tuberculosis   of   infants   and    the    niihary   and 
submiliary  forms  of  the  disease. 

1.      INF.\NTILE    TUBERCULOSIS 

The  tuberculosis  of  infants  deserves  especial  consideration,  as  it 
generally  differs  in  its  pathology,  clinical  course,  and  prognosis  from 
other  forms  of  the  disease.     For  tliis  reason  we  consider  it  separately. 

Occurrence. — Tuberculosis  is  very  frequent  in  infancy.  The 
difficulties  of  diagnosis  and  the  lack  of  autopsies  render  statistics  of 
doubtful  value  as  to  its  frequency.  Among  532  autopsies  of  infants, 
I  have  met  with  tuberculosis  36  times  as  follows: 


Age. 

Number 
of  Autopsies. 

Tuberculous. 

Non-                 Percentage 
Tuberculous.      of  Tuberculous. 

i.T4 
101 

r, 

13 
17 

271             1              2.2 

141              1               N.4 

M             1             16.8 

Total 

532 

30             1            496                          6.8 

From  the  above  table  we  see  that  tuberculosis  is  uncommon 
during  the  first  few  months  of  life.  Generally  more  time  is  required  to 
cause  death.  The  second  quarter  of  the  first  year  contains  a  greater 
number,  and  the  second  half  year  shows  nearly  17  per  cent,  of  tuber- 
culous cases.  My  figures  are  rather  below  the  average,  e.g.,  10  per  cent, 
the  percentage  of  tuberculosis  given  by  Finkelstein  for  the  first  year. 
Tliis  is  due  to  the  pecuUar  nature  of  my  material,  wliich  includes  many 
premature  babies,  cases  of  sepsis,  etc.  [These  figures  cannot  be  regarded 
as  shoTOng  the  frequency  of  tuberculosis  in  infancy,  as  it  is  now  known 
that  tuberculosis  may  be  present,  especially  in  the  cervical,  bronchial, 
or  mesenteric  lymph-nodes  and  not  manifest  itself  macroscopically  or 
even  microscopically.  The  only  exact  test  is  the  biological  one,  namely, 
injecting  the  suspected  material  into  guinea-pigs.  It  is  certain  that  the 
above  figures  would  be  still  larger  if  such  latent  cases  were  included. — H.] 

Etiology. — In  tliis  connection  we  must. refer  to  what  has  been 
written  above.  However  we  must  emphasize  the  fact  that  the  infant  is 
very  susceptible  to  infection  immediately  after  birth.  Frequently  we 
can  trace  the  source  of  the  infection  to  a  parent  or  to  the  nurse.  Milk 
does  not  play  an  important  role  in  the  genesis  of  the  disease  as  it  is 
generally  cooked  previous  to  being  used. 

Pathological  Anatomy. — The  characteristic  of  tuberculosis  of 
infancy  is  the  rapid  generalization  of  the  disease.     I  haA'e  never  seen 


TUBERCULOSIS  593 

a  case  come  to  autopsy  where  the  tuberculosis  was  localized  in  one  organ. 
In  the  early  stages  there  occurs  a  dissemination  by  means  of  the  blood, 
due  to  the  breaking  down  of  a  primary  lesion  which  involves  the  intima 
of  a  vessel,  or  to  a  focus  rupturing  into  a  neighboring  blood  vessel. 

The  type  of  tuberculosis  is  miliary,  submiliary,  or  nodular,  such  as 
we  find  in  the  monkey.  The  latter  is  due  to  the  rapid  growth  and  early 
confluence  of  the  miliary  tubercles.  The  tissue  of  the  infant  is  evidently 
an  excellent  culture  medium  for  the  baciUi.  There  is  also  a  striking 
lack  of  reactive  changes  surrounding  the  lesions.  The  lungs  and  the 
bronchial  lymph-nodes  are  regularly  the  most  involved.  The  former 
besides  showing  mihary  and  nodular  tuberculosis  are  often  the  seat  of 
an  acute  tuberculous  pneumonia.  The  conglomerate  tubercles  often  break 
down  and  form  cavities,  wliich  are  by  no  means  exceptional  (7  in  20 
cases,  Gei pel).    In  fact  we  may  find  true  sequestra;  lying  within  the  cavities. 

The  bronchial  lymph-nodes  and  spleen  are  also  regularly  involved. 
The  hver,  mesenteric  lymph-nodes,  and  intestines  are  very  frequently 
affected.  The  tonsils  (7  in  17  cases),  stomach,  meninges,  bones,  etc., 
are  not  rarely  the  seat  of  tuberculosis. 

Symptoms. — The  symptoms  of  the  tuberculosis  of  infants  are 
manifold  and  variable.  They  present  notliing  characteristic  of  the 
disease.  The  general  appearance  of  an  infant  suffering  from  tubercu- 
losis is  that  of  a  mild  marasmus.  One  of  the  first  symptoms  is  that  the 
child  ceases  to  gain  in  weight.  On  the  other  hand,  we  do  not  always 
find  a  marked  emaciation;  on  the  contrary,  some  children,  especially 
those  in  the  first  months  of  hfe,  remain  fairly  well  nourished.  Indeed 
we  must  remember  that,  under  appropriate  diet,  the  infant  afflicted 
with  tuberculosis  may  even  gain.  Tliis  I  have  repeatedly  seen  demon- 
strated in  nursing  infants.  When  they  begin  to  have  persistent  fever 
and  cavities  develop  in  the  lung  we  find  a  sudden  loss  of  weight.  Not 
rarely,  however,  the  disease  takes  a  rapid  course  and  the  cliildren  die 
before  they  have  lost  materially  in  weight. 

The  appetite  is  generally  lessened  in  the  early  stages.  The  ac- 
customed food  is  refused  and  in  spite  of  all  efforts  the  infant  v,i\\  not 
take  its  nourishment  regularly.  In  some  cases,  enlargement  of  the 
cervical  lymph-nodes  or  of  those  situated  about  the  oesophagus  may 
cause  pain  in  swallowing  (see  Plate  32  c). 

The  fever  is  not  of  a  regular  tj'pe,  and  may  throughout  the  disease  be 
absent  or  at  least  remain  very  slight.    The  following  illustrates  this  point : 

M.  B.,  born  July  7th,  admitted  on  August  lOth,  1901,  for  ocular 
blennorrhoea  and  perforation  of  the  left  cornea.  Died  on  September 
17th,  1901.  Mother  had  tuberculosis.  During  the  infant's  39  days 
stay  in  the  hospital  its  temperature  remained  almost  normal  (see 
Fig.  148).  Weight  on  admission  2330  Gm.,  falhng  to  2000  Gm..  on  arti- 
ficial feeding,  and,  as  the  result  of  changing  to  human  milk,  gradually 

11—38 


594 


THE   DISEASES   OF   CHILDREN 


reaching  2310  Gm.  on  the  day  of  lier  death.  No  pulmonary  symptoms, 
no  cough.  Sudden  death  ^^dth  symptoms  of  collapse.  Autopsy  showed: 
submiUary,  miliary,  and  larger  tubercles  in  the  lungs,  beginning  tuber- 
cular pneumonia,  cheesy  bronchial  lymph-nodes,  miliary  tuberculosis 
of  the  spleen,  hver  and  iddneys,  tuberculosis  of  the  retroperitoneal 
lymph-nodes. 

Nevertheless,  continued  taking  of  the  temperature,  especially 
when  it  is  done  every  two  hours,  is  of  value  in  making  the  diagnosis. 
We  find  that  the  variations  day  by  day  are  greater  than  we  are  accus- 
tomed to  note  in  children.  At  one  time  the  rectal  temperature  will  be 
subfebrile,  at  another  it  will  be  97°  F.  In  other  cases  the  temperature 
may  suddenly  shoot  up  to  102°  F.  or  even  liigher,  and  may  thus  give 
the  alarm.    In  the  last  stages  of  the  disease  we  generally  find  an  irreg- 


Fio. 

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Temperature  curve  uf  ;i  child  (M.  li.)  durnig  tlie  la:^t  3S  days  of  lier  life  <rcctai  Icinpcralure). 

ular  remittent  fever  corresponding  to  the  involvement  of  new  areas. 
We  frequently  meet  with  a  continuous  fever  in  tubercular  pneumonia. 

The  skin  of  tuberculous  infants  often  presents  a  striking  appearance. 
It  is  frequently  of  exceptional  dryness,  so  much  so  that  tliis  may  be 
the  first  sign  to  awaken  suspicion  as  to  the  nature  of  the  illness.  Again 
we  may  find  tuberculids,  lichen  scrofulosorum,  scrofuloderma,  and 
especially  a  tuberculous  folhcuHtis,  which  even  in  infants  may  present 
the  appearance  of  a  sluggish  furunculosis. 

Of  diagnostic  importance  are  the  small  nodules,  not  as  large  as  the 
head  of  a  pin,  situated  beneath  the  cutis,  most  often  in  the  umbihcal 
region.  These  may  also  be  present  in  an  atrophic  condition  not  depen- 
dent on  tuberculosis.  I  have  seen  tuberculous  ulcers  about  the  rectum 
mistaken  for  syphilis.  Careful  examination  of  the  skin  cannot  be  too 
strongly  urged,  as  it  very  frequently  offers  important  diagnostic  aid. 

Examination  of  the  lungs  reveals  varying  conditions.     We  may  find 


TUBERCULOSIS  595 

nothing  but  a  slight  tympany  over  both  lungs,  accompanied  by  some 
intensification  of  the  respiratory  murmur.  This  may  be  expected  in 
miliary  tuberculosis  where  there  is  a  diffuse  dissemination  of  tubercles. 
If  the  tubercles  conglomerate,  and  there  is  a  definite  invasion  of  the 
pulmonary  tissue,  we  find  shght  dulness  and  especially  a  sense  of 
resistance  on  percussion.  Ausculation  reveals  diminished  or  slightly 
broncliial    breathing. 

Where  a  pneumonia,  tuberculous  or  otherwise,  is  superadded  to  the 
miliary  tuberculosis,  we  hear  fine  crepitant  or  subcrepitant  rales,  which 
may  be  more  or  less  coarse  according  to  the  extent  of  involvement  of 
the  broncliial  tubes.  Not  rarely,  especially  at  the  onset  of  a  miliary 
tuberculosis  we  may  hear  diffuse  coarse  rhonchi. 

Cavities  may  be  formed  without  evincing  symptoms.  However, 
at  times  I  have  been  able  to  diagnose  even  small  cavities.  I  have  often 
heard  loud  bronchial  breathing  and  the  so-called  "cracked-pot"  sound, 
which  changes  in  pitch  when  the  mouth  is  opened.  At  times  the 
excellent  conductivity  of  the  infant's  chest  wall  leads  us  astray  in 
interpreting  the  locahzation  of  the  lesions. 

Pleurisy  is  frequently  found,  as  we  should  expect  considering  that 
it  is  almost  always  involved  in  mihary  tuberculosis;  it  is  frecjuently 
of  the  fibrinoiis  variety,  leading  to  early  adhesions.  This  accounts  for  the 
fact  that  we  rarely  meet  with  true  empyemata.  Such  areas  as  break 
into  the  pleural  space  become  at  once  encapsulated,  so  that  pleuritic 
friction  sounds  are  of  short  duration. 

Respiration  is  always  accelerated  in  extensive  tuberculosis;  we 
may,  however,  not  infrequently  find  a  moderate  degree  of  cyanosis. 
Cough  may  be  practically  absent.  At  the  onset  there  is  frequently  a  dry 
hacking  cough;  where  there  are  greatly  enlarged  bronchial  lymph-nodes 
we  may  have  attacks  of  cougliing  resembling  whooping-cough. 

Infants  do  not  expectorate.  By  tickhng  the  epiglotis  with  the 
fingers  we  can  excite  a  fit  of  cougliing,  so  that  by  swabbing  out  the 
entrance  of  the  larynx  we  may  obtain  some  sputum  for  examination. 
We  do  not  see  the  greenish  yellow  expectoration  which  we  are  accus- 
tomed to  find  in  adults.  Even  where  cavities  exist  we  cannot  expect 
to   find   characteristic  sputum. 

The  digestive  tract  in  many  cases  offers  no  symptoms.  Digestion 
progresses  normally,  althougli  where  there  is  marked  intestinal  involve- 
ment we  may  have  diarrhoea  which  persists  in  spite  of  breast-feeding. 
Palpation  of  enlarged  and  cheesy  mesenteric  lymph-nodes  is  possible 
only  in  case  of  greatly  emaciated  cliildren.  Tabes  mesenterica  is  rare 
in  infancy.  When  we  do  find  enlarged  mesenteric  lymph-nodes  these 
are  frequently  not  tuberculous  but  due  to  other  causes. 

The  spleen  is  generally  enlarged  and  extends  below  the  free  border 
of  the  ribs  for  two  fingers  or  more.    The  diagnostic  importance  of  this 


596  THE    DISEASES   OF   CHILDREN 

phenomenon  is  lessened  both  on  account  of  the  frequency  of  enlarge- 
ment of  the  spleen  in  infancy  and  its  frequent  lack  of  enlargement  in 
tuberculous  individuals.  The  liver  is  likewise  not  infrequently  enlarged, 
either  on  account  of  simple  stasis,  or  through  tuberculous  involvement; 
in  the  latter  case  we  may  expect  icterus. 

The  ears  are  often  involved  in  the  tuberculosis  of  infants.  We 
meet  with  peristent  and  intractable  otitis,  multiple  perforation  of  the 
drum,  loosening  of  the  ossicles  and  marked  destruction  of  the  bony 
structures  of  the  inner  ear. 

The  urine  may  be  normal;  but  generally  towards  the  end  of  the 
disease  the  kidneys  become  involved.  We  may  then  find  tubercle 
baciUi  in  the  urine,  and  almost  always  albumin  and  casts.  However, 
tubercle  bacilh  may  be  present  without  involvement  of  the  kidneys. 

In  spite  of  the  rather  frequent  involvement  of  the  meninges  and  even 
of  the  brain,  cerebral  symptoms  are  not  prominent.  True  meningitic 
plienomena  are  rare  in  infants.  Tubercles  of  the  chorioid  are  occasion- 
ally seen.  [Chorioidal  tubercles  do  not  generally  appear  until  the  miliary 
tuberculosis  is  well  advanced.  In  some  cases  they  alone  may  enable  the 
chnician  to  make  the  diagnosis  as  to  the  nature  of  the  meningitis. — H.] 

Diagnosis. — The  diagnosis  may  be  estabhshed  from  the  symp- 
toms described  above.  Tuberculosis  occurs  at  every  age  and  must  always 
be  borne  in  mind. 

The  history  should  be  given  sufficient  weight.  A  consideration  of 
the  general  condition  of  the  cliild  combined  with  that  of  the  physical 
examination  does  not  frequently  permit  of  a  rapid  and  certain  diagnosis. 
As  regards  the  physical  signs,  I  consider  only  one  pathognomonic, 
namely,  the  finding  of  a  pulmonary  cavity.  It  is  frequently  difficult  and 
sometimes  impossible  to  demonstrate  the  presence  of  tubercle  bacilli. 

The  bacilli  should  be  sought  for  patiently  and  repeatedly.  If  there  is 
alack  of  expectoration,  the  method  mentioned  above  may  be  resorted  to. 

The  urine  and  stools  should  be  examined  in  this  regard.  Sometimes 
we  may  find  tubercle  bacilh  in  the  cerebrospinal  fluid,  in  the  absence 
of  all  signs  of  meningitis. 

Where  there  is  no  fever,  tubercuhn  should  be  used  to  determine 
the  diagnosis.  Especially  in  infancy  may  this  test  be  resorted  to,  for 
the  prognosis  is  so  poor  that  we  do  not  need  to  fear  doing  any  harm, 
and,  in  the  second  place,  young  children  bear  large  doses  of  tubercuhn 
well.  The  temperature  generally  falls  quickly  to  the  normal,  as  we 
usually  are  deaUng  with  uncomphcated  cases.  Where  fever  is  present 
tubercuhn  cannot  of  course  be  used. 

In  the  differential  diagnosis,  typhoid  fever  must  be  considered,  as 
where  this  disease  is  prevalent  it  is  very  commonly  met  with  among 
infants,  and  pursues  an  atypical  course.  The  Widal  reaction  must  be 
resorted  to  in  order  to  estabhsh  a  diagnosis.     Chronic  bronchitis  and 


TUBERCULOSIS  597 

chronic  pneumonia  may  simulate  tuberculosis;  besides,  I  have  fre- 
quently seen  empyema  mistaken  for  tuberculosis.  However,  in  tuber- 
culosis we  do  not  find  dulness  of  such  marked  intensity;  in  case  of 
doubt  the  needle  will  decide.  On  the  other  hand,  the  other  chronic 
pulmonary  diseases  can  be  differentiated  only  if  the  patient  improves 
markedly  or  if,  on  the  contrary,  we  find  tubercle  bacilli  in  the  excreta. 
Finally,  there  are  certain  forms  of  sepsis  that  resemble  tuberculosis. 
Furthermore,  a  toxic  intestinal  catarrh,  if  seen  only  for  a  short  period  and 
without  the  aid  of  a  reliable  history,  may  be  mistaken  for  tuberculosis. 

Sometimes  the  tuberculosis  of  infants  proceeds  without  symptoms, 
and  sudden  death  occurs,  the  cause  of  wliich  is  unsuspected  before 
autopsy. 

Prognosis. — The  prognosis  of  tuberculosis  in  infancy  is  bad. 

We  do  not  know  of  a  single  case  which  resulted  in  cure.  Indeed 
there  is  not  a  positive  instance  where  a  tendency  toward  the  limita- 
tion of  the  disease  was  observed.  The  tissues  of  the  infant  do  not 
seem  to  be  able  to  respond  by  protective  inflammatory  reaction.  The 
result  is  that  the  lesions  do  not  become  circumscribed  nor  calcified. 
The  youngest  child  whose  organs  showed  processes  which  may  be  char- 
acterized as  defensive  in  nature  was  one  15  months  old.  In  this  case  a 
zone  of  inflammation  with  the  formation  of  new  vessels  surrounded  the 
tuberculous  area  (Plate  33). 

The  duration  of  the  disease  is  very  variable.  It  may  progress 
rapidly  and  towards  the  end  assume  a  foudroyant  form  as  we  see  in 
the  acute  miliary  type  of  adults.  It  may  progress  slowly,  lasting  months. 
Therefore  it  is  best  not  to  venture  to  make  a  statement  as  to  the 
possible  duration  of  the  disease  even  when  the  diagnosis  is  certain. 

Treatment. — We  can  use  only  prophylactic  measures,  as  there  is 
no  treatment  for  the  disease.  The  two  main  prophjdactics  are  breast- 
feeding and  improvement  of  the  congested  dwellings  of  the  poor. 

We  can  hardly  combat  the  individual  symptoms  successfully.  If  the 
fever  is  liigh,  and  the  infant  restless,  we  should  resort  to  hydrotherapy, 
in  the  form  of  packs  or  lukewarm  baths.  The  best  antipyretic,  should 
we  wish  to  give  one  to  an  infant,  is  pyramidon  given  as  follows: 

Pyraniidon,  1.0-2.0  Gm.  (15-30  gr.),  syrup,  30  c.c.  (1  oz.),  aquae 
ad  100  c.c.  (3  oz.)  one  half  to  one  teaspoonful  every  hour  until  the 
temperature  falls.  Convulsions  may  make  one  wish  to  ]irescribe  nar- 
cotics; in  such  an  event,  chloral  hydrate  0.5  Gm.  to  100  c.c.  (7 J  gr. 
to  3  oz.)  may  be  given  l)y  rectum. 

2.    .\CUTE    MILI.iRY    TUBERCULOSIS 

MiUary  tuberculosis  resembles  an  acute  infectious  disease,  and 
occurs  at  every  stage  of  clrildhood.  We  meet  with  it  in  infants  present- 
ing the  same  picture  as  in  adult  life. 


598  THE   DISEASES   OF   CHILDREN 

Etiology  and  Pathogenesis. — Miliary  tuberculosis  is  almost  never 
primary  in  origin.  It  follows  some  tuberculous  focus  in  the  body, — most 
commonly  cheesy  broncliial  lymph-nodes.  The  primary  focus  may  be 
recent  or  of  long  standing.  Every  person  who  harbors  tubercle  bacilli 
runs  the  danger  of  developing  a  fatal  miliary  tuberculosis.  This  develop- 
ment generally  is  spontaneous  but  may  follow  some  operative  procedure. 
All  that  is  necessary  is  that  the  bacilh  obtain  entrance  to  the  blood 
stream  in  large  numbers,  as  readilj'  happens  when  a  cheesy  lymph-node 
ruptures  into  a  vessel. 

The  tubercle  which  causes  the  general  infection  may  lie  in  the  ves- 
sel (see  Fig.  136)  or  as  just  mentioned  may  rupture  from  without  into 
the  blood  or  lymph  stream.  Naturally  the  blood  vessels  of  a  diseased 
lymph-node  are  most  endangered. 

About  S  or  10  days  following  the  dissemination  of  bacilh,  charac- 
teristic tubercles  appear  throughout  the  body.  The  sites  of  predilec- 
tion are  the  serous  surfaces,  as  well  as  the  spleen,  the  lungs,  the  bone 
marrow,  the  Uver,  the  kidneys,  etc.  No  organ  and  no  part  of  an  organ 
is  immune  from  infection.  The  number  of  tubercles  varies,  but  is  gen- 
erally large.  Following  the  primary  dissemination,  there  may  be  a 
second  or  a  third,  and  even  more  if  the  patient  Uves  sufficiently  long. 
Tliis  may  be  deduced  from  the  various  sizes  and  stages  of  development 
of  the  tubercles  which  we  find  at  autopsy. 

Symptoms. — The  disease  may  set  in  acutely  without  any  pro- 
dromal stage,  and  attack  an  apparently  healthy  cliild.  At  other  times 
the  child  complains  of  indefinite  symptoms  for  a  week  or  more,  is 
apathetic,  lacks  appetite,  and  may  have  an  occasional  rise  of  temper- 
ature, until  suddenly  the  seriousness  of  the  ailment  begins  to  impress 
itself  upon  us.  The  fever  quickly  becomes  marked  but  presents  no 
characteristic  curve. 

In  some  cases  the  fever  rises  gradually,  and  remains  high  with 
remission  of  1  or  2  degrees  until  death.  However  it  may  begin  with  a 
rise  to  104°  F.  or  over,  and  be  characterized  by  marked  and  repeated 
remissions.  The  pulse  is  rapid  in  comparison  with  the  temperature. 
If  the  meninges  are  involved  early  in  the  disease,  we  may  find  a  slow 
or  irregular  pulse  at  the  onset. 

Percussion  and  auscultation  of  the  lungs  afford  little  aid  in  the 
diagnosis.  At  times  there  is  a  slight  tympanitic  note.  Bronchitic 
rales  point  to  involvement  of  the  lungs.  The  breathing  is  rapid  and  a 
considerable  degree  of  c3'anosis  may  be  present. 

The  cough  may  be  very  distressing  and  dry;  in  small  children, 
however,  it  is  frequently  absent  or  of  no  moment.  Expectoration  when 
present  is  scanty.  The  spleen  is  almost  always  enlarged  and  is  hard  and 
firm  in  consistency.  The  urine  at  times  contains  tubercle  bacilh  and 
frequently  gives  the  diazo  reaction.    Involvement  of  the  meninges  may 


TUBERCULOSIS  599 

lead  to  early  cerebral  symptoms.  Indeed  these  may  dominate  the 
clinical  picture  to  such  a  degree  that  one  considers  the  case  one  of 
tuberculous  meningitis. 

The  difference  between  tuberculous  meningitis  and  mihary  tuber- 
culosis with  meningeal  involvement  is  generally  not  clearly  defined. 
We  should  speak  of  tuberculous  meningitis  when  the  miliary  tubercles 
are  confined  to  the  meninges  with  but  one  or  at  most  a  few  tuberculous 
foci  in  the  entire  body.  In  true  mihary  tuberculosis  there  is  to  a  cer- 
tain extent  a  general  dissemination  of  tubercles.  In  tuberculous  men- 
ingitis the  picture  is  distinctly  cerebral  in  type;  in  the  mihary  form, 
death  frequently  intervenes  before  the  cerebral  symptoms  are  very 
marked.  Mihary  tuberculosis  is  equally  prevalent  throughout  all 
stages  of  childhood,  tuberculous  meningitis  is  most  common  between 
the  ages  of  two  and  six. 

Diagnosis. — The  diagnosis  of  mihary  tuberculosis  is  certain  only 
when  we  find  tubercule  bacilh.  This  however  is  not  possible  in  most  of 
the  cases,  for  the  sputum  does  not  contain  bacilh,  as  the  foci  do  not 
generally  communicate  with  the  bronchi.  Sometimes  we  may  find 
tubercle  bacilh  in  the  urine,  but  failure  to  find  them  does  not  affect 
the  diagnosis. 

The  demonstration  of  bacilh  by  inoculation  of  the  blood  into 
animals  requires  too  long  a  period  to  render  it  of  practical  importance. 
Rarely  there  may  be  a  sufficient  number  of  bacilli  in  the  blood  to  per- 
mit us  to  find  them  in  blood  spreads.  We  may,  however,  find  the  bacilh 
in  the  cerebrospinal  fluid,  especially  in  the  characteristic  web,  in  the 
absence  of  all  cerebral  symptoms.  Failure  of  the  fluid  to  react  to 
Fehhng's  test  points  to  an  inflammatory  process,  generally  tuberculous 
in  nature. 

The  tubercuhn  test  is  of  value  only  early  in  the  disease,  on  account 
of  the  temperature  in  the  later  stages.  Examination  of  the  fundus  of  the 
eye  should  never  be  omitted,  as  sometimes  chorioid  tubercles  are  found. 

Of  the  diseases  which  may  offer  difficulty  as  regards  differential 
diagnosis  we  must  place  typhoid  fever  in  the  first  rank. 

At  times  diagnosis  is  impossible,  especially  as  typhoid  fever  so 
often  runs  an  atypical  course  in  childhood.  For  example  it  may  show 
no  continued  fever,  on  the  other  hand  this  is  also  true  of  mihary  tuber- 
culosis. I  would  never  rely  on  the  temperature  to  differentiate  between 
these  diseases.  The  large  spleen  and  the  diazo  reaction  are  common 
to  both,  although  absence  of  the  latter  points  rather  against  typhoid 
fever.  Even  the  roseola  may  be  present  in  miliary  tuberculosis.  The 
lungs  do  not  serve  to  differentiate  the  diseases.  Involvement  of  the 
pericardium  and  pleura  early  in  the  disease,  or  meningeal  symptoms, 
points  rather  to  tuberculosis.  The  Widal  reaction  if  positive  decides 
in  favor  of  typhoid  fever. 


600  THE   DISEASES   OF   CHILDREN 

Next  to  typhoid  fever,  cryptogenetic  sepsis  offers  most  difficulty 
in  the  differential  diagnosis.  Here  again  the  temperature,  spleen,  and 
general  symptoms  do  not  aid  us.  Chills  and  marked  variation  of  the 
temperature  point  to  sepsis,  but  are  of  rare  occurrence.  The  diazo 
reaction  points  to  tuberculosis;  on  the  other  hand,  haemorrhages  of 
the  skin  or  mucous  membrane  favor  the  diagnosis  of  sepsis. 

I  once  saw  a  case  of  sinus  thrombosis  with  such  indefinite  symp- 
toms that  it  was  regarded  as  miliary  tuberculosis  to  the  very  end. 

Lobular  pneumonia  and  capillary  bronchitis  may  be  mistaken  for 
miUary  tuberculosis  or  vice  versa.  In  this  regard  we  should  consider 
that  in  the  latter  disease  the  rapidity  of  respiration  and  dyspnoea  are 
in  marked  contrast  to  the  intensity  of  the  pulmonary  symptoms.  The 
diazo  reaction  speaks  in  favor  of  tuberculosis. 

I  have  also  seen  severe  influenza  with  meningeal  involvement 
resembling  miliary  tuberculosis. 

Localized  tubercidosis  of  the  bronchial  lymph-nodes  with  caseous 
formation  may  cause  difficulty  in  diagnosis. 

Prognosis. — The  prognosis  of  mihary  tuberculosis  is  bad.  In 
pronouncing  the  diagnosis  you  doom  the  cliild  to  death.  The  disease 
lasts  a  varying  period.  Death  may  ensue  after  8  to  10  days  or  it  may 
be  postponed  for  4,  6,  or  even  more  weeks. 

Treatment. — There  is  none.  We  may  give  symptomatic  treatment; 
chloral  hydrate  as  mentioned  above,  or  cold  compresses  or  ice  bags  to 
relieve  headache.  Hydrotherapy,  prolonged  baths  at  30-32°  C.  (86- 
90°  F.)  often  quiet  the  patient.  Free  access  of  fresh  air  should  be 
allowed  in  order  to  relieve  the  dyspnoea.  The  use  of  oxygen 
inhalations    may   be   indicated. 

B.     LOCALIZED  TUBERCULOSIS 
1.    TUBERCULOSIS    OF    THE    BRONCHIAL    LYMPH-NODES 

The  bronchial  glands  in  cliildren  arc  certainly  a  locus  minoris 
resistentia*.  The  bacilli  must  here  gain  an  easy  entrance  and  furthermore 
must  flourish  at  this  site.  TFe  flnd  the  bronchial  lymph-nodes  almost 
without  exception  involved  in  every  tubercidous  child.  In  very  many  cases 
we  can  easily  demonstrate  that  these  areas  are  the  oldest  in  point  of 
origin,  and  that  the  spread  of  the  disease  throughout  the  system  or  to 
the  lungs  or  meninges  originated  from  this  focus. 

It  is  questionable  whether  isolated  bronchial  lymph-node  tuber- 
culosis is  as  common  as  one  would  imagine  from  a  superficial  post-mor- 
tem examination.  A  more  careful  examination  will  often  disclose  small 
areas  in  the  lungs  wliich  are  easily  overlooked.  However,  the  main 
feature,  from  an  anatomical  as  well  as  from  a  clinical  point  of  \aew, 
is  the  tuberculosis  of  the  broncliial  lymph-nodes,  as  the  small  pulmonary 
foci,  even  if  primary,  are  frecpiently  completely  healed. 


PLATE  32. 


':    •-    ^ 

;'  =   3 


■   i-  ■>  .=  ti: 

:  — ,   >•.  •=  •= 


«?, 


•5  *■  >  S 


C     V    C   pS 


S      t:- 


i      2  J  •-  S  . 


:  a  -  s 


i  .3 

"d  "u  .f 


-"  =5  t-      . 


TUBERCULOSIS 


601 


On  the  other  hand  we  have  unquestionable  cases  of  isolated  tuberculosis 
of  the  bronchial  lymph-nodes,  indeed  Weleminsky  has  lately  expressed 
the  opinion  that  glandular  involvement  always  precedes  involvement 
of  the  organ  itself,  even  in  the  case  of  the  lung  and  its  lymph-nodes. 

A  careful  consideration  of  the  anatomical  relationship  is  necessary 
in  order  to  understand  this  question.    The  Vjronchial  lymph-nodes  com- 


FlG.  149. 


ThjTOid  gland 
01.  submaxill. 
Trachea 
Asc.  aort; 
Gl.  track. -tjesophajj. 
Gl.  subdavical. 

Gl.  mediast.  ant 

Vena  cava  superior. 

Gl.  track. -branch. 

Gl.  pulmonales.' 

Bronclli 

Gl.  pleiiroputmon.. 

Thoracic  dud. 

Lung 


^t.  auric,  ant. 

Gl.  auric,  post. 

Gl.  )ugul.  sup. 

.Gl.  cervic.  superfic.  (fore  part) 
Left  subclavian  vein 
Gl.  supraclaviadarea 


Left  l>-mphatic  trunk 

■Gl.  azill. 

Left  main  bronchus 
-Gl.  between  great 
■Desc.  aorta  vessels 
„,      ,  ,  ,      and  pen- 

■Gl.  pleura  costalea  cardium 

'Pericardium 

<Epophagu3 
Gl.  lymph,  slemi 
Diaphragm 


Bronchial  and  other  lymph-nodes  mainly  affected  in  tuberculosis.     (The  dark-colored  markmgs  represent 
the  most  superficial,  the  light-colored  the  most  deeply  situated  nodes.) 


prise  those  lymphatic  glands  in  the  thorax  which  receive  the  lymph 
stream  from  the  lungs  and  the  bronchial  system.  As  these  nodes,  how- 
ever, are  closely  connected  wth  other  lymphatic  glands  of  the  thorax 
and  the  neck,  we  must  consider  them  as  well.  There  are  three  sets  of 
bronchial   lymph-nodes: 

(a)  The  tracheobronchial  lymph-nodes  situated  at  the  bifurcation 
of  the  trachea,  one  of  which  is  situated  in  the  angle  formed  by  the  sepa- 
ration of  the  main  bronchi. 


602  THE    DISEASES   OF   CHILDREN 

(6)   The  bronchial  lytnph-nodes  situated  along   the   main  bronchi. 

(c)  The  pulmonary  lymph-nodes  situated  at  the  hilus  of  the  lungs, 
also  peribronchial  in  tlieir  arrangement,  and  extending  to  or  into  the 
parenchyma. 

All  three  groups  receive  their  lymph  from  the  lungs,  the  bronchi 
and,  in  part,  from  the  posterior  aspect  of  the  heart.  Their  enlarge- 
ment leads  most  especially  to  pressure  upon  the  trachea,  the  larger  and 
middle  sized  bronchi,  as  well  as  upon  the  recurrent  laryngeal  nerve. 

Other  lymph-nodes  to  be  considered  are: 

(d)  The  anterior  mediastinal,  about  12  in  number,  situated  pos- 
terior to  the  sternum  and  around  the  large  vessels,  e.g.,  in  the  space 
between  the  right  innominate  artery,  in  the  concavity  below  the  right 
subclavian  artery,  and  in  the  concavity  formed  by  the  arch  of  the  aorta. 

According  to  Friedleben,  the  nodes  lying  below  the  concavity  of 
the  right  subclavian  are  very  frequently  affected.  The  lymph  enter- 
ing these  nodes  comes  from  the  anterior  portion  of  the  diaphragm  and 
from  the  upper  surface  of  the  liver,  as  well  as  from  the  pericardium, 
the  heart  and  the  thymus.  Their  enlargement  leads  to  compression  of 
the  great  vessels. 

(e)  The  posterior  mediastinal  lymph-nodes  situated  along  the  aorta 
and  a?sophagus  and  receiving  the  lymph  from  the  a'sophagus,  poste- 
rior part  of  the  diaphragm,  pericardium  and  the  liver.  Enlargement 
produces  compression  of  the  oesophagus,  or  even  of  the  aorta.  All  these 
nodes  (a-e)  are  connected  by  anastomosis,  so  that  disease  of  one  set 
may  be  transmitted  to  another.  They  also  connect  with  nodes  lying 
outside  of  the  thoracic  cavity,  of  which  the  most  important  groups  are: 

(/)  The  tracheal  and  cesophageal  lymph-nodes.  These  are  covered 
by  the  sternothyroid  muscle,  and  extend  from  the  isthmus  of  the  thy- 
roid gland,  down  along  the  anterior  aspect  of  the  trachea,  two  or  three 
being  situated  somewhat  to  the  left  on  the  oesophagus. 

(g)  The  jugular  lymph-nodes  lying  beside  the  internal  jugular  veins. 

(h)  The  supraclavicular  lymph-nodes  lying  above  the  clavicle  and 
between  the  borders  of  the  trapezius  and  the  sternomastoid  muscles. 

(i)  The  superficial  cervical  lymph-nodes  lying  on  the  upper  half  of 
the  sternomastoid  muscle  and  below  and  beliind  the  external  ear. 

(j)  The  submaxillary  glands  situated  behind  the  chin. 

All  these  nodes  (/-/)  communicate  directly  or  indirectly  not  only 
among  each  other  but  also  icith  the  true.broncJiial  nodes. 

[Tliis  important  fact  is  not  definitely  established.  Recent  experi- 
mentors  have  been  unable  to  demonstrate  by  means  of  the  injection  of 
colored  fluid  into  the  cervical  lymph-channels  any  connection  between 
the  cervical  and  bronchial  lymph-nodes.  Again  cUnical  experience 
shows  that  we  may  frequently  meet  with  tuberculosis  of  the  cervical 
lymph-nodes  without  a  secondary  involvement  of  the  bronchial  lymph- 


TUBERCULOSIS  603 

nodes  or  lungs.  It  also  shows  that  of  the  many  cases  of  pulmonary 
tuberculosis  in  which  the  broncliial  lymph-nodes  are  affected,  few  are 
comphcated  by  a  tuberculosis  of  the  cer\ncal  lymph-nodes.  These  cir- 
cumstances, experimental  as  well  as  clinical,  must  be  weighed  when 
we  judge  of  the  existence  and  importance  of  the  anastomosis  between 
the  cervical  and  thoracic  lymph  systems. — A.  F.  H.] 

The  question  as  to  how  the  tubercle  bacilli  gain  admission  to  the 
bronchial  lymph-nodes  allows  of  two  answers:  either  they  gain  entry  to 
the  lungs  by  means  of  the  venous  blood  current  or  by  the  air  and  are 
carried  thence  to  the  lymph-nodes  by  the  interlacing  lymph  capillaries, 
or  they  follow  the  direct  path  from  lymph-nodes  outside  the  thorax, 
more  especially  the  cervical  chain.  We  must  also  not  overlook  the  fact 
that  following  a  tuberculosis  of  the  bronchial  lymph-nodes  a  retrograde 
current  may  be  set  in  motion  which  will  bring  about  the  involvement 
of  nodes  situated  external  to  the  thoracic  cavity. 

Symptoms. — The  clinical  picture  of  tuberculosis  of  the  bronchial 
lymph-nodes  is  by  no  means  clear  if  we  except  those  rare  cases  where 
the  entire  symptom-complex  points  to  the  diagnosis. 

The  onset  of  the  disease  is  generally  insidious.  The  condition  of 
the  child  passes  almost  imperceptibly  from  one  of  health  to  that  of 
disease.  The  appetite  becomes  poor,  the  cheeks  lose  their  color,  the 
child  soon  grows  thin  although  it  continues  to  grow  in  length,  which 
makes  its  loss  of  flesh  appear  more  marked.  Irregular  pyrexia  at  this 
time  points  to  some  systemic  affection,  but  examination  generally  dis- 
closes nothing.  In  fact  the  lack  of  cause  for  the  change  in  the  general  con- 
dition of  the  child  is  truly  characteristic  of  tuberculosis  of  the  bronchial 
lymph-nodes. 

In  other  cases  the  course  may  be  a  different  one.  After  a  short 
prehminary  stage,  high  fever  may  set  in,  the  temperature  remaining  at 
40°  C.  (104°  F.)  for  weeks  and  gradually  falling  by  lysis. 

The  fever  curve  is  atypical,  it  may  be  broken  by  marked  remis- 
sions or  it  may  be  continuously  high.  We  meet  with  all  the  manifes- 
tations which  accompany  prolonged  fever  in  children:  loss  of  weight 
and  strength,  lack  of  appetite,  apathy,  etc. 

In  those  cases  where  the  nodes  are  much  enlarged  and  form  a  large 
cheesy  mass,  we  may  be  able  to  diagnose  the  condition  bj-  the  physical 
signs,  however,  in  this  regard  I  am  somewhat  sceptical,  as  are  also 
Widerhofer  and  Henoch.  Dulness  can  hardly  ever  be  absolutely  deter- 
mined as  due  to  enlargement  of  bronchial  nodes.  Even  large  tumors  maj' 
not  cause  dulness,  owing  to   the  resonance   of  the   pulmonary  tissue. 

Two  areas  are  especially  accessible  for  percussion,  the  interscapu- 
lar space  at  the  level  of  the  2nd  and  3rd  dorsal  vertebra^,  and  anteriorly 
over  the  manubrium  sterni.  In  these  locations  an  increased  sense  of 
resistance  may  also  aid  us  in  the  diagnosis. 


604  THE   DISEASES   OF   CHILDREN 

Auscultation  generally  reveals  notliing  on  account  of  the  excellent 
conductivity  of  the  neighboring  pulmonary  tissue.  In  the  interscapular 
space  according  to  Seitz,  rough  harsh  respiration,  especially  in  expiration 
may  be  heard ;  Widerhofer  states  that  this  is  more  marked  on  the  left  side. 

We  should  always  try  to  feel  a  resistance  deep  down  in  the  supra- 
sternal space.  Sometimes  a  mass  of  nodes  may  be  palpated.  Palpa- 
tion of  the  cervical  and  submaxillary  nodes  may  also  be  of  aid  in  the 
diagnosis.  However  these  nodes  may  not  be  enlarged  in  spite  of,  or 
notwithstanding,  tuberculosis  of  the  bronchial  nodes. 

The  cough  is  an  important  symptom  and  may  be  characteristic. 
It  occurs  in  prolonged  attacks,  occurring  perhaps  at  intervals  of  hours, 
and  frequently  resembles  the  paroxysms  of  pertussis.  However  there 
is  no  mucous,  no  vomiting,  no  regular  nightly  exacerbations.  The 
attacks  of  cougliing  are  probably  caused  by  pressure  of  the  enlarged 
nodes  upon  the  vagi  nerves. 

We  must  now  consider  what  may  be  designated  as  the  indirect 
consequences  of  tuberculosis  of  the  bronchial  lymph-nodes  caused  by 
compression  on  the  important  organs  in  their  immediate  vicinity  (see 
Plate  32,  Fig.  c).  In  the  first  place  the  trachea  is  exposed  and  may  be 
almost  flattened  out  by  pressure.  The  result  is  a  disturbance  of  res- 
piration, dyspnoea,  cj'anosis  and  finally  suffocation.  If  the  nodes  press 
upon  only  one  bronchus,  more  or  less  of  the  lung  may  cease  to  func- 
tionate. Again,  the  oesophagus  may  be  entirely  occluded  by  pressure 
from  without,  resulting  in  pain,  difficulty  in  deglutition,  leading  even  to 
starvation.  The  blood  vessels,  more  especially  the  large  veins  may  be 
compressed,  thus  giving  rise  to  all  the  symptoms  of  venous  stasis  (see 
Plate  32  c). 

Course. — The  course  of  tuberculosis  of  the  bronchial  lymph-nodes 
is  variable.  The  disease  may  remain  stationary;  the  cheesy  nodes  may 
become  encapsulated  or  calcified.  Nevertheless  such  a  tuberculous  node 
existing  in  the  human  body  cannot  be  disregarded,  especially  if  the 
affected  individual  be  a  child.  For  at  any  time  an  accident  or  an  inter- 
current disease,  especially  measles  or  whooping-cough,  but  also  scarlet 
fever  or  diphtheria,  may  hght  up  the  latent  focus  and  endanger  the 
entire  body.  The  bacilli  may  be  transmitted  to  the  lungs,  to  the 
meninges,  or  throughout  the  body. 

In  other  cases,  tuberculosis  of  the  bronchial  lymph-nodes  may 
lead  by  continuity  or  by  a  retrograde  current  to  pulmonary  infection, 
resulting  in  a  tuberculous  peribronchitis,  a  diffuse  tuberculous  pneu- 
monia, or  in  a  local  process  in  the  neighborhood  of  the  cheesy  nodes, 
especially  those  at  the  liilus  of  the  lungs  (see  Plate  32,  Fig.  a). 

The  nodes  may  break  into  the  surrounding  organs.  I  have  seen  a 
sudden  rupture  into  the  trachea  lead  to  the  expectoration  of  masses 
of  cheesy  material;    a  rupture  into  the  bronchi   cause  an  aspiration 


TUBERCULOSIS  605 

pneumonia;  a  rupture  into  the  vessels  lead  to  a  miliary  tuberculosis 
of  the  lungs  or  entire  body.  The  a-sophagus,  pericardium,  or  pleural 
cavity  may  hkcwise  be  penetrated.  Sooner  or  later  in  almost  all  these 
cases  death  ensues. 

Diagnosis. — The  diagnosis  in  the  early  stages  is  difficult.  If,  after 
many  thorough  examinations  of  a  child,  we  are  unable  to  find  the  seat 
of  the  disease,  we  may  suspect  the  bronchial  lymph-nodes  to  be  the  source 
of  the  trouble.  Radioscopy  sometimes  enables  the  diagnosis  in  advanced 
cases.     Tubercuhn  may  be  tried  in  some  cases  when  there  is  no  fever. 

The  typical  paroxysms  of  cough  are  of  diagnostic  value  where  per- 
tussis can  be  excluded.  In  advanced  cases  with  stenosis  of  the  trachea, 
where  no  history  of  the  slow  onset  is  obtainable,  it  may  be  difficult  to 
differentiate  the  disease  from  diphtheria  or  even  from  a  foreign  body  in 
the  bronchus.  Sudden  onset  of  the  disease  with  high  fever  may  cause  it 
to  be  mistaken  for  miliary  tuberculosis. 

Prognosis. — The  prognosis  is  not  bad  so  long  as  the  focus  is  not 
too  large  and  there  is  no  caseation.  Where  cheesy  degeneration  has  taken 
place,  we  generally  have  an  unfavorable  outcome,  due  to  involvement  of 
other  organs. 

Treatment. — The  prophylaxis  of  tuberculosis  of  the  broncliial 
lymph-nodes  is  that  of  tuberculosis  in  general.  The  treatment  consists  in 
improving  the  general  condition  of  the  child,  wluch  at  times  accomplishes 
a  great  deal.  A  diet  rich  in  fat,  varied  in  nature,  containing  a  large 
quantity  of  fruits  and  vegetables,  is  to  be  recommended,  and  its  results 
tested  by  regular  weigliing  of  the  patient.  The  appetite  may  be  stimu- 
lated by  giving  spicy  articles  of  diet,  or  by  arsenic,  especially  in  the  form 
of  the  natural  arsenical  waters. 

Mud  baths  are  to  be  recommended.  Some  recommend  ^ving  potas- 
sium iodide  or  inunctions  of  iodine- vasogen.  A  careful  tuberculin  cure  in 
the  case  of  patients  mth  normal   temperature  is  by  no  means  futile. 

In  addition,  we  should  resort  to  symptomatic  treatment.  Con- 
tinued fever  ma}^  sometimes  be  held  in  check  for  a  long  while  by  means 
of  hydrotherapy,  lactophenin,  pyramidon  or  aspirin.  When  the  nodes 
compress  the  oesophagus  we  should  be  most  careful  in  the  use  of  the 
stomach  tube.  At  times  the  symptoms  improve  following  rupture  of  the 
glands.  Where  a  marked  dyspncca  results,  resort  to  a  low  tracheotomy 
and  to  the  removal  of  the  glands  from  the  trachea  may  be  indicated. 

A  few  words  may  be  added  in  tliis  connection  concerning  tuber- 
culosis of  the  cervical  lymph-nodes.  This  is  of  frequent  occurrence, 
at  times  following  involvement  of  tlie  bronchial  nodes,  but  far  oftener 
through  infection  from  the  mouth,  from  carious  teeth,  from  scrofulous 
eruptions  about  the  mouth  and  nose,  etc.  Compared  to  the  enlarge- 
ment of  these  nodes  through  pyogenic  processes,  the  tuberculous  involve- 
ments progress  slowly.      They  may  be  merely  as  large  as  a  kernel  of 


606  THE    DISEASES    OF   CHILDREN 

rice  or  a  cherry  but  may  also  reach  the  size  of  a  plum  or  become  even 
larger.  If  these  nodes  tend  to  infect  those  more  centrally  situated, 
early  operative  removal  should  be  considered.  As  long  as  the  affected 
nodes  remain  isolated,  only  dietetic  measures  should  be  resorted  to.  At 
times  these  nodes  reach  such  a  stage  of  caseation,  that  the  neck  becomes 
virtually  imbedded  in  cheesy  masses.  The  reproduction  in  Plate  32 
conveys  an  idea  of  this  condition. 

2.    TUBERCULOSIS    OF    THE    LUNGS 

(a)   Tuberculous  Pneumonia  • 

Tuberculous  pneumonia  may  follow  immediately  upon  specific 
infection  of  the  lungs,  or,  as  is  more  commonly  the  case,  result  from 
the  accidental  hghting  up  of  a  subacute  or  chronic  tuberculous  process. 
This  condition  represents  anatomically  an  exudation  into  the  alveoh, 
caused  by  the  vital  processes  of  the  tubercle  bacilli  and  its  toxines. 
The  pneumonic  area  may  be  miliary,  lobular,  or  lobar  in  extent.  The 
peculiar  glassy  appearance  of  the  cut  section  of  tliis  form  of  pneumonia 
leads  to  its  appellation  of  gelatinous.  The  rapid  caseation  of  these 
processes  point  to  their  specific  nature. 

The  clinical  symptoms  resemble  those  of  a  simple  pneumonia.  If 
the  areas  are  scattered,  a  lobular  pneumonia  may  be  simulated;  on  the 
other  hand,  the  sudden  onset,  cliill,  high  fever,  and  physical  signs  may 
suggest  a  lobar  pneumonia. 

The  course  may  be  either  a  very  rapid  one,  resulting  in  death  after 
a  few  days,  following  continuous  fever,  marked  dyspnoea,  and  terminal 
cardiac  weakness,  or,  when  caseation  takes  place,  death  after  a  few 
weeks,  following  high  and  irregular  temperature  and  a  great  loss  of  flesh 
and  strength.    A  complicating  pleurisy  is  not  uncommon. 

A  differential  diagnosis  from  simple  pneumonia  is  frequently  very 
difhcult.  A  liistory  of  previous  tuberculous  disease  is  very  important. 
At  times  the  absence  of  herpes  and  of  rales  redux  and  all  signs  pointing 
to  resolution  may  allow  us  to  exclude  a  simple  pneumonia.  In  this  form, 
as  in  the  lobular  variety,  the  demonstration  of  the  tubercle  bacillus 
chnches  the  diagnosis. 

Treatment  is  powerless  in  this  disease.  Absolute  rest  in  bed  must 
be  required  and  symptomatic  treatment  resorted  to.  The  dyspnoea  may 
be  reheved  for  a  time  by  oxygen  inhalations.  Morpliine,  codeine,  etc., 
should  be  employed  to  allay  the  cough.  Digitahs  frequently  relieves 
the  subjective  symptoms. 

(b)  Miliary  and  Submiliary  Tubemdosis  of  the  Lungs 

This  sometimes  remains  localized  in  the  lungs.  In  such  cases  the 
symptoms  are  the  same  as  we  have  described  where  the  lungs  are 
involved  in  a  general  mihary  tuberculosis. 


PLATE  33. 


^SSSSBSiS 


TUBERCULOSIS  C07 

(c)  Chronic  Tuberculosis  of  the  Lungs  {Phthisis  pulmonum) 

Whereas  phthisis  is  very  rare  during  the  first  years  of  life,  and  never 
occurs  in  infancy,  we  occasionally  meet  with  it  after  the  fourth  or  fifth 
year,  and  from  then  on  it  occurs  with  ever  increasing  frequency  as  we 
approach  puberty.  Its  characteristics  are  a  chronic  course  and  the 
reactive  indurative  processes  of  the  surrounding  tissue. 

Its  pathogenesis  is  not  certain.  The  majority  of  authors  believe 
in  an  aerogenic  infection,  others  however  in  a  haematogenic  origin. 
The  former  believe  that  the  lungs  are  first  invaded,  the  latter  that 
the  pulmonary  infection  is  merely  secondary  to  tuberculosis  of  the 
bronchial  glands.  On  the  other  hand,  Behring  upholds  an  alimentary 
origin  also  for  this  form  of  tuberculosis,  and,  as  I  have  said  above, 
I  favor  this  view. 

There  is  no  unanimity  as  to  the  meaning  of  the  phthisical  habitus 
(see  Figs.  135-141)  which  we  have  described  above.  Some  consider  it 
merely  a  physical  type  predisposing  to  tuberculous  infection,  while  others 
believe  it  to  be  an  expression  of  an  existing  tuberculosis.  The  latter 
view  is  strengthened  by  the  fact  that  we  find  tuberculosis,  often  latent, 
so  frequently  among  children.  According  to  tliis  interpretation  Freund's 
opinion  that  the  lack  of  development  of  the  first  rib  predisposes  to 
pulmonary  tuberculosis,  would  have  no  basis. 

Symptoms  and  Course. — The  on.set  is  gradual,  accompanied  by  a 
loss  of  flesh  and  strength,  perhaps  a  cough,  but  an  absence  of  all  physical 
signs.  In  the  early  stage,  the  variations  in  the  temperature,  if  this  is 
taken  at  two  hour  intervals,  may  serve  to  indicate  the  nature  of  the 
trouble.  It  may  be  months  before  definite  pulmonary  symptoms  are 
manifested.  Meanwhile,  the  cough  or  expectoration,  if  the  latter  be 
present,  is  generally  not  characteristic. 

At  times  an  observant  physician  may  be  able  to  diagnose  the  con- 
dition before  there  are  definite  changes  to  be  discovered  in  the  lungs, 
by  finding  tubercle  bacilli  in  the  sputum.  At  times  we  may  be  able  to 
hear  dry  or  harsh  rales  in  the  morning,  always  in  the  same  area,  and 
percussion  may  reveal  scattered  tympanitic  foci.  However,  the  excel- 
lent conductivity  of  the  thorax  in  children  renders  tliis  difficult.  We 
must  remember  that  in  childhood  the  apices  of  the  lungs  are  by  no  means 
so  frequently  the  earliest  parts  to  be  involved  by  tuhercxdosis  as  is  the  case 
in  adult  life.  The  first  focus  may  quite  as  well  be  in  some  other  area. 
But  as  we  approach  puberty  we  find  that  tuberculosis  has  a  tendency 
to  attack  the  apices  first. 

In  spite  of  frequent  and  long  remissions,  the  child  gradually 
develops  a  phtliisis  which  renders  a  diagnosis  simple.  AVe  see  the  char- 
acteristic flattening  of  the  chest,  and  the  loss  of  weight,  which  however 
mav  not  be  marked  if  the  fever  is  not  high  and  a  mixed  infection  has 


608  THE   DISEASES   OF   CHILDREN 

taken  place.  The  temperature  shows  irregular  rises.  The  lungs  give 
signs  of  scattered  foci  of  tuberculosis,  not  necessarily  involving  the 
apices  at  first.  Areas  of  dulness  or  of  tympanitis  are  found  and  on 
auscultation  persistent  catarrhal  signs  are  heard,  especially  in  the  morning, 
the  rales  being  sticky  in  character. 

Later  cavities  may  develop,  as  the  affected  areas  break  down;  this 
takes  place  readily  and  rapidly  in  cliildhood  (see  Plate  31).  They  mani- 
fest the  same  signs  as  in  adults,  namely,  a  tympanitic  or  "cracked-pot'' 
note,  rough  bronchial  breathing,  and  loud  metaUic  rales.  Tliis  is  es- 
pecially striking  when,  after  a  fit  of  cougliing,  accompanied  by  profuse 
greenish  yellow  expectoration,  particularly  in  the  morning,  we  find  the 
above  signs  over  an  area  where  previously  there  existed  dulness  and 
absent  respiratory  sounds.  Concomitant  with  the  formation  of  cavities, 
we  see  marked  failing  in  the  general  condition  of  the  patient,  and  ir- 
regular fever,  although  in  cliildren  apyrexia  may  exist  almost  until 
death.  Numerous  comphcations  may  now  set  in,  and  the  patient  gen- 
erally dies  of  an  acute   tuberculous  pneumonia  or  of  gradual  inanition. 

Of  the  numerous  comphcations  just  mentioned,  hsemoptysis  is 
relatively  rare,  whereas  pleurisy  is  frequent.  We  often  find  a  dry 
pleurisy  at  the  onset,  or  even  pleurisy  with  effusion,  in  fact  the  fluid  in 
the  early  stages  may  be  purulent  or  hsemorrhagic. 

In  older  children  the  larynx  is  by  no  means  rarely  involved.  The 
intestinal  canal,  subjected  as  it  is  to  the  swallowed  sputa,  frequently 
contains  tuberculous  ulcers,  which  cause  a  troublesome  diarrhoea  and 
sap  the  strength  of  the  patient. 

Diagnosis. — The  diagnosis  of  early  tuberculosis  is  very  important, 
as  upon  tliis  the  question  of  therapeutic  aid  frequently  rests.  In  tills 
connection  the  family  history  is  of  importance  and  the  possibility  of 
exposure  to  infection.  We  must  remember  that  this  may  have  taken 
place  years  previous  to  the  onset  of  the  disease.  An  examination  of  the 
lungs  should  be  repeatedly  made,  and  we  should  note  whether  the 
catarrhal  signs  are  always  found  in  the  same  areas.  Well  circumscribed 
pulmonary  affections  are  ahvays  stispi'cious.  Repeated  noting  of  the  tem- 
perature mil  often  give  evidence  of  disease.  The  weight  should  be 
followed  carefully.  Sputum  examination  should  be  made  repeatedly. 
We  may  even  resort  to  attempts  at  causing  the  bacilh  to  multiply  in 
the  sputum  or  to  injecting  it  into  guinea-pigs.  In  the  examination  of  the 
sputum  especial  attention  should  be  paid  to  the  presence  of  elastic  fibres. 

Tuberculin  may  be  used  if  fever  is  absent.  After  its  injection  the 
rales  often  become  more  moist  and  bacilh  may  be  found  in  the  sputum. 

Prognosis.— The  prognosis  is  not  bad  if  the  disease  is  in  an  early 
stage.  The  fact  that  it  has  assumed  a  chronic  form  and  has  not  devel- 
oped into  an  acute  mihary  tuberculosis,  points  to  a  relative  resistance 
of  the  cliild  or  to  a  lack  of  virulence  of  the  bacilh.       Indeed  we  fre- 


TUBERCULOSIS  609 

quently  meet  with  a  local  cure,  although  permanent  cures  are  seldom 
encountered.  After  some  years,  between  the  ages  of  20  and  30,  during 
the  period  of  pregnancy,  or  as  the  result  of  some  harmful  employment, 
the  spark  is  generally  kindled  anew  and  the  supposedly  healed  focus 
gives  I'ise  to  a  fatal  infection. 

Treatment. — Insipient  t ubercido.nx  i.s  a  curable  disease.  We  should 
treat  suspected  cases  in  the  same  way  as  those  in  which  the  diagnosis  is 
definitely  established.  Phthisical  children  should  not  attend  school,  both 
for  their  own  welfare  and  for  the  sake  of  the  other  pupils;  with  the  excep- 
tion of  schools  instituted  especially  for  those  afflicted  with  tuberculosis. 

Hygienic  and  dietetic  measures  are  of  primary  importance.  The 
atmosphere  should  be  free  from  dust;  sea  air  is  pi'cferable  in  the  early 
stages  and  liigh  altitudes  for  all  curable  forms  of  the  disease.  We  can 
expect  little  from  a  residence  of  a  few  weeks.  If  relapses  are  to  be  pre- 
vented, years  must  be  spent  in  a  favorable  climate.  Judicious  harden- 
ing is  a  necessary  part  of  the  cure.  As  much  time  as  possible  should  be 
spent  out  of  doors.  An  especially  valuable  form  of  treatment  consists  in 
having  the  children  he  in  the  open  air,  well  covered  and,  if  necessary, 
warmed  by  hot  water  bags.  Sports,  if  not  overdone,  are  also  of  benefit, 
even  in  the  winter  time.  Respiratory  exercises  and  gymnastics  carried 
out  under  supervision  should  be  a  part  of  the  daily  routine.  In  pre- 
scribing the  diet,  increase  of  flesh  is  the  most  important  consideration, 
and  in  tliis  connection  we  should  not  pay  so  much  attention  to  the 
quantity  the  patient  consumes  as  to  the  quantity  he  utiUzes. 

I  prefer  a  chet  rich  in  fats,  such  as  cream,  butter,  bacon,  given 
with  plenty  of  fresh  vegetables,  and  raw  fruit.  Good  raw  milk  is  to  be 
recommended,  but  all  alcoholic  beverages  are  to  be  avoided. 

The  following  regime  is  an  example  of  the  kind  to  be  recommended 
for  cases  of  incipient  tuberculosis:  at  7  a.  m.  a  cup  of  cocoa  with  cream, 
a  roll  with  butter  and  some  scraped  ham  should  be  given  to  the  patient 
in  bed.  A  half  hour  later  the  child  should  rise  and  take  respirator}^  or 
gymnastic  exercises.  An  hour  pre^^ous  to  the  next  meal  he  should  he 
down,  preferably  in  the  open  air.  At  10  a.  m.  a  second  meal  consisting 
of  an  egg  and  bacon,  rye  bread,  a  raw  apple,  pear  or  grapes  should  be 
given.  Following  tliis  he  should  once  more  take  exercise  and  rest  in 
the  open  air.  A  half  hour  before  lunch  he  should  take  about  two  ounces 
of  beef  juice  or  concentrated  bouillon.  The  lunch  may  consist  of  rare 
meat,  green  vegetables.  At  4  o'clock  the  patient  is  given  tea  or  coffee 
with  cream,  and  bread  with  butter,  honey  or  marmalade.  Finally  at 
half  past  seven  he  has  a  Ught  supper,  a  cereal,  or  omelet  with  preserves. 

Exact  regulations  as  regards  sleep,  rest,  emploj-ment,  recreation, 
diet  and  exercise  have  also  their  psycliical  value.  Drugs  may  be  given  to 
increase  the  nutrition,  such  as  arsenic  or  at  times  iron  preparations. 
I  do  not  use  such  as  are  supposed  to  affect  the  tuberculous  process,  as 

11-39 


610  THE    DISEASES   OF   CHILDREN 

for  example  creosote;  however,  this  may  be  given  with  double  the  quan- 
tity of  tincture  of  gentian.  It-is  a  good  ride  to  avoid  unnecessary  drugs  so 
as  not  to  disturb  the  appetite. 

Tuberculin  is  being  used  as  a  therapeutic  measure,  and  has  been 
especially  recommended  by  Ganghofner. 

We  shoukl  begin  with  very  small  doses,  1/1000-1/100  of  a  mg.  and 
increase  only  when  we  obtain  no  reaction.  First  we  should  preceed 
by  doubling  the  dose,  and  later  by  increasing  by  0.1-0.2  mg.  until  0.01 
Gm.  is  borne  without  reaction. 

Symptomatic  treatment  is  necessary  in  the  incurable  cases.  Here 
too  we  should  make  use  of  the  open  air  treatment,  as  it  is  very  comfort- 
ing to  the  patient.  AVe  know  of  very  little  to  counteract  the  fever;  at 
times  lukewarm  baths  serve  this  purpose,  or  large  doses  of  pyramidon, 
three  grains  hourly  until  the  temperature  falls.  The  disagreeable 
sweats  are  combated  by  sponging  with  dilute  \'inegar  and  then  apply- 
ing a.  simple  powder,  or  one  containing  formalin.  We  do  not  need  to 
resort  to  the  use  of  atropine  except  in  extreme  instances,  but  we  make 
use  of  the  following  proscri]ition: 

R      Pyramidon  camphorici -5.0 oiss 

Syrupi 35.0 oioi 

Aquae  ad 100.0 Biiio" 

M.S.— 2-3  teaspoonfuls  at.  half  Iiourly  intervals  before  retiring. 

The  best  treatment  for  haemoptysis  is  rest,  which  is  best  obtained 
by  means  of  morphine.  In  advanced  cases  narcotics  (morphine,  codeine) 
should  not  be  spared,  e.g.: 

R      Morphinse  hydrochlor 0.01-0  03 gr.  J-J 

Syrup  althaea) 35.0 5i5i 

Aquse  ad 100.0 Siiioii 

M.S. — Teaspoonful  or  lialf  teaspoonful  doses  according  to  the  age  of  the  child. 

To  older  children  morphine  is  given  in  powdered  form;  the  dose  is 
^  mg.  for  each  year  of  hfe: 

R      Morphinaj  hydrochlor 0.015 gr.  J 

Sacharri 2.5 gr.  xl 

M.    Fiat  pulvis.    Divide  in  parts  V. 

S. — One  powder  at  night. 

The  dose  of  codeine  is  three  times  that  of  morphine. 

For  diarrhoea,  tannigen  or  tannalbin  may  be  used. 

3.    TUBERCULOUS    PLEURISY 

Pleurisy  may  accompany  any  form  of  tuberculosis  and  should 
always  be  thought  of  in  this  connection.  It  may  be  of  any  variety,  and 
is  frequently  the  initial  appearance  of  the  disease  in  older  children. 
The  physical  signs  and  temperature  do  not  aid  in  the  diagnosis.  If 
the   exudate   shows   no   bacteria  the   process  is  probably  tuberculous. 


TUBERCULOSIS  611 

A  careful  examination  of  the  sediment  or  coagulum  of  the  fluid  will 
reveal  tubercle  bacilh  far  oftener  than  is  generally  suj)posed.  As  a 
final  resort  we  may  inject  the  fluid  into  the  peritoneal  cavity  or 
lactating  breast   of  a  guinea-pig. 

A  predominance  of  mononuclear  leucocytes  is  said  to  favor  the 
diagnosis  of  tuberculosis.  It  is  certain  that  the  presence  of  ha'mor- 
rhagic  exudate  points  to  this  disease. 

We  may  relieve  the  pain  by  warm  applications,  by  painting  the 
thorax  with  tincture  of  iodine,  followed  by  applications  of  ointment, 
or  by  10  per  cent,  iodvasogen.  Serous  exudate  should  be  removed 
by  aspiration,  and  purulent  exudates  by  large  incisions.  The  use  of 
other  therapeutic  measures  is  only  of  value  where  the  process  is  not 
tuberculous,  e.g.,  the  salicylates  in  rheumatic  pleurisy.  In  general  the 
tuberculosis  should  be  treated  as  above  outlined. 

4.    TUBERCULOSIS    OF    THE    LARYXX 

I  have  never  seen  a  primary  tuberculosis  of  the  larynx;  secondary 
infection,  however,  is  common  in  older  children.  TMs  complication  is 
evidenced  by  a  roughness  or  hoarseness  of  the  voice.  Laryngoscopic 
examination  shows  a  profuse  redness  and  swelling  about  one  or  more 
ulcers  or  tubercles.  The  pain  is  frequently  marked  and  interferes 
with  swallomng  and  nutrition.  The  cough  may  be  very  troublesome. 
The  ulcers  can  be  confused  only  with  sypluUtic  affections.  However 
the  presence  of  the  pulmonary  condition  in  tuberculosis  as  well  as 
the  sharply  circumscribed  nature  of  the  syphilitic  ulcers  serve  to  differ- 
entiate the  two. 

Treatment  consists  of  mild  applications  of  lactic  acid.  Cocaine 
is  used  to  relieve  the  pain. 


Index 


PAGE 

Abdominal  typhus  (see  Typhoid  fever)  426 

Abrasions  in  the  newborn 1 

Acidosis  in  diabetes 222 

Adenoid  tissue  in  late  hereditary  syphilis  548 

Albuminuria  in  diphtlieria 385 

in  influenza 454 

in  scarlet  fever 273 

in  the  newborn 14 

Ana-mia  after  haemorrhage 133 

after  illness 134 

at  the  end  of  the  nursing  period.  .  .  .    140 

in  malnutrition 134 

of  school  children 135 

symptoms 136 

treatment 137 

pernicious 154 

anatomy 156 

symptoms 1 55 

treatment 147 

pseudoleuksemia  infantum 140 

etiology 141 

pathology 142 

prognosis 146 

symptoms 143 

treatment 147 

symptomatic 133  - 

Anteconceptional  syphilis 503 

Antitoxin  in  diphtheria 400,  402 

dosage 404,  407 

sickness  after 407 

success  of 406 

technic 402 

Arteriosclerosis      in     late      hereditary 

syphilis 548 

Arthritis,  chronic 493 

diagnosis 497 

treatment 498 

gonorrhceal 490 

scarlatinal 491 

septic 489 

syphilitic 492 

varicellosa 342 

Articular    rlieumatism    (see    Rheuma- 
tism)    481 

Asphyxia 99 

acquired 102 

etiology 103 

symptoms 103 


PAGE 

Asphyxia,  acquired,  treatment 104 

congenital 99 

etiology 99 

prognosis 101 

symptoms 100 

treatment 102 

Asthenic  pneumonia 254 

Atelectasis  (see  Asphyxia) 99 

Banti's  disease 167 

Barlow's  disease  (see  Infantile  scurvy)  180 

Baths  in  scrofula 239 

Birth  injuries 1 

swellings 5 

Blood  in  ansemia  pseudoleukaemia  in- 
fantum   145 

in  chlorosis 148 

in  leukaemia 160 

physiology  of 131 

in  pernicious  amcmia 156 

in  pseudoleukaemia 164 

in  purpura 174 

in  typhoid 467 

in  whooping-cough 467 

Bone  marrow,  affections  of 168 

Bones,  affections  of,  in  scrofula 235 

injuries  to,  in  newborn 3 

lesions  of,  in  syphilis 522,  550 

lesions  of,  in  late  hereditary  sj'philis  544 

Bronchial  glands,  tuberculosis  of  .  .398,  470 

Bronchiectasis  in  whooping-cough 470 

Bronchitis  in  measles 247,  264 

Bronchopneumonia  in  measles 253 

Caput  succedaneum 4 

Carbohydrates  in  diabetes 221 

Cephalaematoma 5 

complications 6 

diagnosis 8 

internum 7 

pathogenesis 7 

pathology 7 

treatment 9 

Cerebral  haniorrhages  in  the  newl)orn  2 

Chicken-pox  (see  Varicella) 330 

Chlorosis 147 

diagnosis 150 

symptoms 148 

613 


614 


INDEX 


PAGE 

Clilorosis,  treatment 151 

Circulatory  system  in  wliooping-cough    471 

CoUes'  law  in  syphilis 502,  504,  559 

Conceptional  syphilis. . .    503 

Condylomata  in  syphilis 541,  543,  556 

Congenital    syphilis     (see     Hereditary 

sypliilis) 500 

Conjunctival  diphtheria 383,  398 

treatment 412 

Convalescence  after  mealses 261 

Convulsions  in  the  newborn 2 

in  whooping-cough 471 

Coryza  in  measles 262 

in  sj-philis  (see  Rhinitis) 513 

Cranial  bones  in  the  newborn 3 

Craniotabes  in  rachitis 200 

Croup  during  measles 253 

Debility,  congenital SI 

anatomy 85 

body  temperat  ure 83 

diagnosis 88 

etiology 82 

feeding  in 94 

incubators  in 90 

mortality 89 

occurrence 81 

physiology,  pathology 83 

prognosis 88 

treatment 90 

weight 84 

Desquamation  in  scarlet  fever 270 

Development,  disturbances  of,  in  hered- 
itary syphilis 550 

Diabetes  insipidus 225 

symptoms 225 

treatment 226 

mellitus 219 

etiology 220 

prognosis 222 

symptoms 221 

treatment 223 

Diarrhcea  in  typhoid  fever 432 

Diazo  reaction  in  measles 255 

Diet  in  diphtheria 409 

in  rheumatism 488 

in  scarlet  fever 409 

in  typhoid  fever 442 

Diphtheria 335 

anatomy 365 

bacillus 357 

conjunctival 383 

diagnosis 389 

differential  diagnosis 391 

etiology 357 

exanthems  in 385 

extension  to  respiratory  organs 373 


page; 

Diphtheria,  general  treatment  of     ....   408 

heart  failure  in 386 

intubation  in 413 

larj-ngeal 382 

localized  pharyngeal 367 

local  treatment  of 410 

nasal 380 

pathogenesis  of 362 

prognosis  of 398 

progressive  pharjTigeal 369 

prophylaxis  of 399 

secondary 389 

and  scarlet  fever 292 

serum  therapy  in 402 

of  skin-wounds 384 

of  the  stomach  and  intestines 385 

tracheotomj'  in 416 

treatment  of 402 

of  the  vulva 384 

Diphtheritic  paresis  and  paralysis 387 

Diseases  of  the  new^born 1 

of  puberty Ill 

Ductus  omphalomesentericus    (see  Vi- 
telline duct) - 34 

Dysentery ■. 444 

diagnosis 446 

pathology 444 

symptoms 445 

treatment 447 


Ear,  affections  of,  in  influenza 

in  scarlet  fever 270, 

treatment  of 

in  late  hereditary  syphilis 

in  measles 255, 

Eczema  about  nose  in  scrofula 

Endocarditis  in  rheumatism 

in  scarlet  fever 

Epistaxis  in  purpura 

Eruption  in  diphtheria 

in  influenza 

in  measles 249, 

in  scarlet  fever 271, 

in  sypliilis 

differential  diagnosis  of 

in  varicella 

Erj'sipelas  in  the  newborn 

et  iology 

spnptoms 

treatment 

Extrabuccal  infections  in  scarlet  fever  . 

Exudative  diathesis 

Eyes,  affections  of,  in  scrofula 

in  hereditary  syphilis 535, 

in  late  hereditary  syphilis 


453 
277 
314 
544 
263 
233 
484 
287 
177 
385 
455 
254 
281 
512 
552 
335 
53 
54 
53 
55 
277 
228 
231 
540 
544 


Foetal  syphilis 507 


IX 

pa<;e 

Foetal  tuberculosis 571 

Fourth  disease 326 

Furunculosis  in  syphilis 538 

Gastro-enteric  symptoms  in  influenza.  453 

German  measles  and  measles 258 

complications 324 

contagiousness 322 

course 325 

diagnosis 325 

symptoms 323 

treatment 325 

Germinal  transmission  of  tuberculosis  574 

Glycosuria,  transitorj- 219 

Gonorrhoeal  arthritis 400 

Gummata  in  late  hereditary  syphilis  .  .  575 

Hicmatoma  of  the  sternocleidomastoid  9 

occurrence 9 

pathologj- 9 

prognosis 10 

symptoms 9 

treatment 10 

Hiemoglobinuria,  paroxysmal 181 

treatment 185 

H;emopliilia 170 

etiology 171 

heredity 170 

prognosis 172 

sjTnptoms 170 

treatment 183 

Hiemorrhages    in    the    newborn     (see 

Mela?na) 74 

in  typhoid  fever 432 

in  sj-philis 538 

in  whooping-cough ; .  469 

meningeal  and  cerebral 2 

umbilical 43 

vaginal 18 

Hsemorrhagic  affections 169 

diagnosis  of 182 

treatment  of 183 

diathesis 169 

Heart  in  diphtheria 363,  386 

in  influenza 457 

in  measles 255 

in  scarlet  fever 286,  805 

Hereditary  sj-philis 500 

classification 501 

Heredity  in  sj-phiUs 506 

in  tuberculosis 507 

Herpes  in  scarlet  fever 281 

Hutchinson's  triad  in  hereditary  syphilis  553 

Hydrotherapy  in  measles 265 

Icterus  neonatorum 20 

occurrence 21 


DEX 


615 


Icterus,  pathogenesis 22 

pathology 22 

symptoms 20 

Immunity  in  diphtheria 402 

in  scarlet  fever 308 

Incubators 90 

Finkelstein's 92 

Rommel's 92 

Infantile  scurvy 180,  186 

clinical  picture 187 

diagnosis 183,  193 

etiology 191 

history 186 

occurrence 187 

pathology 190 

relations  to  rickets 191 

SjTnptoms 180,  188 

treatment 185,  194 

Influenza 450 

bacteriology 450 

diagnosis 456 

onset 451 

symptoms 452 

t  reatment 457 

Intestinal  tuberculosis 577 

Intestine,  affections  of,  in  measles    .  256,  266 

Intubation 413 

technic 414 

Inunctions  in  treatment  of  syphilis   563,  567 
Iodides  in  the  treatment  of  hereditary 

syphilis 564 

Iron,  use  of,  in  ansemia 151 

Joints,  affections  in  scrofula 235 

in  articular  rheumatism 482 

in  hereditary  syphilis 545 

Kidneys  in  diphtheria 366 

hereditarj'  sj-phihs 533 

Klebs-Loffler  bacillus 357 

Koplik's  spots  in  measles 245 

Larj-ngeal  diphtheria 382 

treatment 413 

Larj-nx,  in  sj'philis 543 

involvement  of,  in  diphtheria 373 

irritation  of.  in  measles 247 

nondiphtheritic  affections  of 396 

tuberculosis  of 611 

Latency  in  tuberculosis 573 

Leukseraia 158 

etiologj- 159 

SjTnptoms 160 

treatment 161 

Liver  in  sj'phiUtic  fcetuses 510 

infants 5;32 

Limgs  in  diphtheria 363 

in  sj'philis 510 


616 


INDEX 


Lungs  in  tuberculosis 584,  595 

Lymph-nodes  in  diplU  heria 363,  365 

in  German  measles. . 324 

in  }iereditary  syphilis 536 

in  late  hereditary  syphilis 549 

in  measles 256 

in  scarlet  fever 270,  279 

treatment  of 315 

in  scrofula 227,  234 

in  tuberculosis 584,  600 

Mammary  glands  in  the  newborn,  se- 
cretion of 16 

Mastitis  in  the  newborn 17 

treatment 18 

Mastoiditis  in  scarlet  fever 278 

Measles 243 

complications 252 

course  of 251 

and  croup 253 

desquamation 251 

diagnosis 257 

and  diphtheria 253,  263 

hydrotherapy  in 265 

Koplik's  spots  in 245 

mode  of  transmission  of 243 

predisposit  ion  t  o 244 


prognosis . 


259 

rash  in 249,254 

without  rash 250 

symptoms 244 

temperature  in 248 

treatment  of 262 

Mela^na  neonatorum 72 

course 74 

diagnosis 78 

treatment 80 

Mercury    in    treatment    of    hereditary 

syphiUs 563 

subcutaneous  administration  of 564 

Mouth  cavity,  diphtheria  of 37 

Mucous  membranes  in  late  hereditary 

syphilis 547 

Mumps 419 

complications 423 

contagiousness 422 

course 422 

diagnosis 425 

etiology 424 

symptoms 419  421 

treatment 425 

Nasal  diphtheria 371,  380 

treatment  of 411 

Navel,  amniotic 31 

arteritis  and  phlebitis  of 38,  41 

catarrhal  inflammation  of 36 


Navel,  cutis 31 

diseases  of 26 

fungus  of 42 

gangrene  of 42 

hiemorrhages  of 43 

hernia  of 31,  46 

inflammation  of 37 

moist  gangrene  of 36 

uses  of 37 

Necrosis  of  pharynx  in  scarlet  fever.  .  .  275 

Nephritis  in  scarlet  fever 298 

treatment 318 

in  varicella 341 

Nervous      symptoms      in      hereditary 

syphihs 534 

in  influenza 454 

in  measles 251,  256,  266 

in  scarlet  fever 294 

in  typhoid  fever 430 

in  varicella 342 

in  whooping-cough 471 

Nose,  affections  of  in  scrofula 233 

scarlet  fever 270,  277 

in  hereditary  syphilis 513 

in  late  hereditary  syphilis 547 

Obstetrical  paralysis 10 

diagnosis 13 

etiology 13 

prognosis 13 

symptoms 10 

treatment 13 

Omphahtis 37 

Open  wounds  in  the  newborn 1 

Ophthalmia  neonatorum 56 

etiology 57 

prophylaxis 58 

symptoms 56 

treatment 69 

Osteomalacia,  relation  to  rachitis 211 

Oza?na  in  late  hereditary  syphilis 547 

Palate  in  late  hereditary  syphihs 548 

Pancreas  in  diabetes  mellitus 220 

Paralysis  (see  Obstetrical) 10 

diphtheritic 387 

in  syphilis 530 

Parasyphilitic  lesions 538 

Paronychia  in  syphilis 516 

Parotitis,  epidemic  (see  Mumps) 419 

Paroxysmal  hsemoglobinuria 181 

etiology 181 

prognosis 182 

symptoms 181 

Peliosis  rheumatica 175 

Pemphigus  syphiliticas  neonatorum .  .  .  518 

Perforation  in  typhoid  fever 432 


INDEX 


617 


PACK 

Pericarditis  in  rheumatism 484 

Pernicious  aniemia 154 

Pertussis  (see  Whooping-cough) 458 

Pharynx  in  late  hereditary  sypliilis. . . .  548 
Placental    transmission   of    tuberculo- 
sis   575 

Pleurisy,  tuberculous 610 

Pneumonia  after  scarlet  fever 296 

after  tuberculosis 606 

Polyuria  in  diabetes  mellitus 220 

Postconceptional  syphilis 503 

Predisposition  to  tuberculosis 575 

Pregnancy,  reactions  in  newborn 16 

Prematurity 81 

Profeta's  law  in  sj'philis 505,  560 

Pseudodiphtheria 394 

Pseudoleukaemia 162 

anatomy 164 

diagnosis 165 

prophylaxis 166 

sjnuptoms 164 

Pseudorheumatism 489 

Puberty,  acute  infectious  diseases  of  .  .  124 

blood  at 120 

circulatory  system  at 121 

digestive  system. in 123 

diseases  of Ill 

hygiene  of 128 

nutrition  during 128 

pathology  of 115 

physiology  of Ill 

thyroid  gland  at 112,  122 

urinary  organs 123 

Purpura 172 

abdominal 178 

etiology 173 

fulminans ISO 

hajmorrhagica 177 

course 178 

rheumatica 175 

simplex 174 

treatment  of 184 

Rachitic  deformities 203 

rosary 200 

Rachitis 196 

clianges  in  thorax 200 

in  pelvis 204 

complications 207 

diagnosis 213 

etiology 212 

pathologic  anatomy 208 

chemistry 210 

sjrmptoms 196 

tarda 211 

time  of  onset 207 

treatment 217 


Radioscopy  in  syphilitic  bone  les- 
ions  526,552 

Rash  in  measles 249 

in  scarlet  fever 249,  254 

absence  of 271,  281,  282 

Respiratory  system  in  influenza 453 

whooping-cougli 469 

Rheumatism,  acute  articular 481 

complicat  ions 484 

diagnosis 486 

etiologj' 481 

symptoms 482 

treatment 487 

chronic  articular 493 

nodular 486,  489 

in  scarlet  fever 288 

Rhinitis  in  hereditarj-  syphilis 513 

Rontgen  ray  (see  Radioscopy) 526 

Rotheln  (see  German  measles) 321 

Rubella  (see  German  measles) 321 

Scarlet  fever 268 

course  in 276 

differential  diagnosis  in 289 

general  clinical  picture  of 268 

immunity  from 308 

inculcation  of 270,  273 

infectious  character  of 309 

initial  symptoms  of 271,  280 

Scars  in  hereditary  syphihs 550,  552 

Sclerema 107 

occurrence 108 

pathogenesis 109 

treatment 110 

Scleroedema 105 

occurrence 106 

pathogenesis 106 

symptoms 105 

treatment 107 

Scorbutus  (see  Infantile  scurvy) 186 

Scrofula 227 

predisposition  to 228 

prognosis 236 

prophylaxis 237 

treatment 238 

Scurvy  (see  Infantile  scurvy) 186 

Sepsis  in  the  newborn 60 

clinical  picture 64 

causes 61 

diagnosis 70 

pathology 69 

portals  of  entry 63 

susceptibility 62 

symptoms 66 

t  rent  ment "1 

Septic  infection  in  sj'philis 537 

Sequelir  of  scarlet  fever 297 


618 


INDEX 


ScqueUr  of  heart  affections 305 

lymphadenitis 302 

nephritis -98 

Serum  rashes  and  measles 258 

sickness 407 

Serum  tlierapj-  in  diphtheria 402 

in  scarlet  fever 315 

in  typhoid 440 

Skin  after  measles 252 

Skin  lesions  in  hereditary 

syphilis 514,  519,  552 

in  late  hereditary  syphilis 546 

in  tuberculosis 584,  594 

Skull,  changes  in  syphihs 531 

Spirocha;ta  palUda 501 

Spleen,  diseases  of 166 

enlargement  of 167,  554 

in  hereditary  syphilis 542,  554 

ptosis  of 167 

in  tuberculosis 595 

Still's  disease 496 

Stools  in  dysentery 446 

in  typhoid  fever 496 

Strabismus  from  diphtheritic  paralysis  388 

Sypliilide,  maculopapular 519 

papulopustular 520 

small  papular 521 

ulcerative 520 

Syphihs 500 

acquired  in  childhood 556 

diagnosis 558 

antenatal,  prophylaxis  in 561 

bone  lesions  in 522 

classification  of  hereditary 501 

diagnosis  of 552 

feeding  in 559 

fcEtal 507 

anatomical  changes  in 508 

death  due  to 511 

from  the  father 502 

from  the  mother 503 

immunity  to  mother  and  fcEtus 504 

in  infancy 512 

late  hereditary 550 

and  maternal  nursing 559 

motor  disturbances  in 529 

nervous  system  in 534 

prognosis  in 554 

relapses  in  early  childhood 540,  556 

skin  lesions  in 514 

transmission  of  hereditary 500,  506 

treatment  of 563 

visual  changes  in 532 

Teeth  in  hereditary  syphilis 551 

Temperature  in  influenza 452 

in  measles 248 


Temperature  in  scarlet  fever 272,  284 

in  typhoid  fever 435 

Tetanus  neonatorum 47 

etiology 49 

sjTnptoms 47 

treatment 51 

Thrush  and  diphtheria 395 

Thymus,  hyperplasia  of 397 

Thyroid  gland,  enlargement  of 398 

Tongue  in  scarlet  fever 272 

Tongue  in  tj-phoid  fever 431 

Tonsillar  abscess  in  scarlet  fever 276 

Tonsillitis  and  diphtheria 372 

witliout  membrane 391 

in  scarlet  fever 270,  274 

Tracheotomy,  technic 416 

Tubercle  bacillus 56S,  578,  585 

bovine  and  human  types 569 

technic  in  examination 585 

Tuljerculiri,  diagnostic  value  of        586 

treatment  in  scrofula 241 

Tuberculosis 568 

acute  miliary 597 

diagnosis 599 

etiology 598 

prognosis 600 

symptoms 598 

treatment 600 

of  bronchial  lymph-glands  in  wlioop- 

ing-cough 470 

course 604 

diagnosis 605 

sj'mptoms 603 

treatment 605 

chronic 607 

diagnosis 608 

prognosis 608 

symptoms 607 

treatment 609 

diagnosis  of 581 

etiology  of 568 

feeding  in 990 

foetal 571 

frequency  of 581 

germinal  transmission  of 574 

lieredity  of 570 

of  infants • 592 

diagnosis 596 

occurrence ' 592 

pathology 592 

prognosis 597 

symptoms 593 

treatment 597 

infection  during  life 576 

intestinal 577 

and  measles 241 

pathogenesis 570 


INDEX 


619 


Tuberculosis,  pathology 578 

physical  sign^i  in 584 

placental  transmission  of 575 

prophylaxis 588 

and  scrofula 227 

treatment 591 

pleurisy  in 610 

Tuberculous  pneumonia 606 

Typhoid  fever 426 

cause 427 

course 429 

diagnosis 438 

mode  of  transmission 426 

pathologj- 428 

prognosis 439 

relapses 437 

sjTTiptoms 429 

treatment 439 

Umbilical  cord,  hernia  of 31 

pathogenesis 32 

prognosis 33 

symptoms 31 

treatment. 33 

Umbilicus(see  Navel) 26 

fungus  of 42 

gangrene 42 

haemorrhages  of 43 

hernia  of 46 

infection  of  blood  vessels 38 

munamification  of 29 

ulcer  of 37 

Urachus  fistulae 35 

Uric  acid,  infarcts  in  the  newborn 14 

Urine  in  diabetes  insipidus 225 

melhtus 219 

Vaccination 348 


FACE 

Vaccination,  technic 350 

treatment 352 

Vaginal  hsemorrhages  in  the  newborn  .      18 

etiology 19 

Varicella 330 

complications  and  seqvielse 341 

contagion 331 

diagnosis 345 

eruption  in 335 

locat  ion  of 339 

immunity  from 332 

incubation 334 

and  scarlet  fever 332 

treatment  of 346 

and  variola  and  vaccinia 333 

Variola,  non-identity  with  varicella  . .  .   333 

^'incent's  angina  and  diphtheria 393 

ViteUine  duct,  diagnosis 34 

persistence  of 34 

prognosis 34 

Vomiting  in  scarlet  fever 280 

^'ulva,  diphtheria  of 384 

treatment  of 411 

Werlhoff 's  disease 177 

Wet-nurse,  sj-philitic  infection  from.  .  .  561 

Whooping-cough 458 

atjTjical  forms 468 

bacteriology' 462 

complications 469 

diagnosis 473 

duration 469 

etiologj- 460 

infection 459,  461 

prognosis 475 

prophylaxis 476 

symptomatology 462 

treatment 477 


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